175 research outputs found
Allogenic hematopoietic cell transplantation in a patient affected by large granular lymphocyte leucemia and multiple sclerosis
No full textSerum ferritin levels and endocrinopathy in medically treated patients with β thalassemia major
No full textThe association between iron overload indices and pathology of the heart and liver in transfusion-dependent patients with β thalassemia major (TM) has been extensively studied. Nonetheless, data on endocrine disease remains limited. This was a cross-sectional study of 382 TM patients treated with regular transfusions and desferrioxamine at the Thalassemia Center in Dubai, UAE. Retrieved data included demographics, splenectomy status, steady-state serum ferritin levels, and the presence of endocrinopathies (diabetes mellitus, hypothyroidism, hypoparathyroidism, and hypogonadism). Multivariate logistic regression analyses were used to determine which variables were independently associated with the occurrence of each endocrinopathy. The mean age of patients was 15.4 ± 7.6 years, with an equal sex distribution. The mean serum ferritin level was 2597.2 ± 1976.8 μg-l. The frequencies of specific endocrinopathies were diabetes mellitus (10.5percent), hypothyroidism (6.3percent), hypoparathyroidism (10.5percent), and hypogonadism (25.9percent). On multivariate logistic regression analysis, patients with a serum ferritin level 2,500 μg-l, but not 1,000-2,500 μg-l, were 3.53 times (95percent CI 1.09-11.40) more likely to have diabetes mellitus, 3.25 times (95percent CI 1.07-10.90) more likely to have hypothyroidism, 3.27 times (95percent CI 1.27-8.39) more likely to have hypoparathyroidism, and 2.75 times (95percent CI 1.38-5.49) more likely to have hypogonadism compared to patients with a serum ferritin level ≤1,000 μg-l. However, splenectomized patients with serum ferritin levels ≤2,500 μg-l had comparably high rates of all endocrinopathies as patients with serum ferritin levels 2,500 μg-l. Endocrinopathy is common in TM patients treated with desferrioxamine therapy, especially in patients with serum ferritin levels 2,500 μg-l or those splenectomized. © 2012 Springer-Verlag.Aessopos A, 2005, ANN HEMATOL, V84, P353, DOI 10.1007-s00277-004-1002-4; Anderson LJ, 2001, EUR HEART J, V22, P2171, DOI 10.1053-euhj.2001.2822; Atichartakarn V, 2003, INT J HEMATOL, V78, P139, DOI 10.1007-BF02983382; Au WY, 2008, HAEMATOL-HEMATOL J, V93, P116, DOI 10.3324-haematol.11768; Aydinok Y, 2011, J PEDIAT HEMATOL ONC, V33, P374, DOI 10.1097-MPH.0b013e31820c34ef; Baskin H Jack, 2002, Endocr Pract, V8, P457; Borgna-Pignatti C, 1998, ANN NY ACAD SCI, V850, P227, DOI 10.1111-j.1749-6632.1998.tb10479.x; Borgna-Pignatti C, 2004, HAEMATOLOGICA, V89, P1187; Brittenham GM, 2011, NEW ENGL J MED, V364, P146, DOI 10.1056-NEJMct1004810; Cappellini MD, 2010, ANN NY ACAD SCI, V1202, P231, DOI 10.1111-j.1749-6632.2010.05548.x; Carpenter JP, 2011, CIRCULATION, V123, P1519, DOI 10.1161-CIRCULATIONAHA.110.007641; Cohen AR, 2008, BLOOD, V111, P583, DOI 10.1182-blood-2007-08-109306; Crary SE, 2009, BLOOD, V114, P2861, DOI 10.1182-blood-2009-04-210112; Cunningham MJ, 2004, BLOOD, V104, P34, DOI 10.1182-blood-2003-09-3167; Davis BA, 2004, BLOOD, V104, P263, DOI 10.1182-blood-2003-08-2841; de Assis RA, 2011, EUR J RADIOL; Delea TE, 2007, TRANSFUSION, V47, P1919, DOI 10.1111-j.1537-2995.2007.01416.x; Eldor A, 2002, BLOOD, V99, P36, DOI 10.1182-blood.V99.1.36; Farmaki K, 2010, BRIT J HAEMATOL, V148, P466, DOI 10.1111-j.1365-2141.2009.07970.x; Farmaki K, 2011, BLOOD CELL MOL DIS, V47, P33, DOI 10.1016-j.bcmd.2011.03.007; Gabutti V, 1996, ACTA HAEMATOL-BASEL, V95, P26; Galanello R, 2010, ANN NY ACAD SCI, V1202, P79, DOI 10.1111-j.1749-6632.2010.05591.x; Gamberini Maria Rita, 2008, Pediatr Endocrinol Rev, V6 Suppl 1, P158; Hershko C, 2010, ANN NY ACAD SCI, V1202, P1, DOI 10.1111-j.1749-6632.2010.05544.x; Jaruratanasirikul S, 2008, EUR J PEDIATR, V167, P873, DOI 10.1007-s00431-007-0602-0; Jensen CE, 1997, EUR J HAEMATOL, V59, P76; Kirk P, 2010, J MAGN RESON IMAGING, V32, P315, DOI 10.1002-jmri.22245; Modell B, 2008, J CARDIOVASC MAGN R, V10, DOI 10.1186-1532-429X-10-42; Musallam K, 2008, PEDIATRICS, V121, pE1426, DOI 10.1542-peds.2007-1944; Nathan DM, 2009, DIABETES CARE, V32, P193, DOI 10.2337-dc08-9025; Noetzli LJ, 2009, BLOOD, V114, P4021, DOI 10.1182-blood-2009-06-225615; OLIVIERI NF, 1994, NEW ENGL J MED, V331, P574, DOI 10.1056-NEJM199409013310903; Phrommintikul A, 2006, HEART, V92, P1467, DOI 10.1136-hrt.2005.079970; Piga A, 2010, ANN NY ACAD SCI, V1202, P75, DOI 10.1111-j.1749-6632.2010.05586.x; Platis Odysseas, 2004, Pediatr Endocrinol Rev, V2 Suppl 2, P279; Restaino G, 2011, MAGN RESON MED, V65, P764, DOI 10.1002-mrm.22640; Rund D, 2005, NEW ENGL J MED, V353, P1135, DOI 10.1056-NEJMra050436; Shalitin S, 2005, EUR J HAEMATOL, V74, P93, DOI 10.1111-j.1600-0609.2004.00371.x; Shoback D, 2008, NEW ENGL J MED, V359, P391, DOI 10.1056-NEJMcp0803050; Skordis N, 2006, EUR J HAEMATOL, V77, P150, DOI 10.1111-j.1600-0609.2006.00681.x; St Pierre TG, 2005, BLOOD, V105, P855, DOI 10.1182-blood-2004-01-0177; TAHER AT, 2009, HEMOGLOBIN, V33, P46; Tavazzi D, 2001, BRIT J HAEMATOL, V112, P48, DOI 10.1046-j.1365-2141.2001.02482.x; Telfer PT, 2000, BRIT J HAEMATOL, V110, P971, DOI 10.1046-j.1365-2141.2000.02298.x; Thuret I, 2010, HAEMATOL-HEMATOL J, V95, P724, DOI 10.3324-haematol.2009.018051; Wang CH, 2006, HEMOGLOBIN, V30, P257, DOI 10.1080-03630260600642609; Wood JC, 2010, ANN NY ACAD SCI, V1202, P123, DOI 10.1111-j.1749-6632.2010.05545.x; Wood JC, 2005, BLOOD, V106, P1460, DOI 10.1182-blood-2004-10-398277
Iron overload and chelation therapy in myelodysplastic syndromes
No full textIron overload remains a concern in MDS patients especially those requiring recurrent blood transfusions. The consequence of iron overload may be more relevant in patients with low and intermediate-1 risk MDS who may survive long enough to experience such manifestations. It is a matter of debate whether this overload has time to yield organ damage, but it is quite evident that cellular damage and DNA genotoxic effect are induced. Iron overload may play a critical role in exacerbating pre-existing morbidity or even unmask silent ones. Under these circumstances, iron chelation therapy could play an integral role in the management of these patients. This review entails an in depth analysis of iron overload in MDS patients; its pathophysiology, effect on survival, associated risks and diagnostic options. It also discusses management options in relation to chelation therapy used in MDS patients and the impact it has on survival, hematologic response and organ function. © 2014 Elsevier Ireland Ltd.Alessandrino Emilio Paolo, 2011, Am J Hematol, V86, P897, DOI 10.1002-ajh.22104; Alessandrino EP, 2010, HAEMATOL-HEMATOL J, V95, P476, DOI 10.3324-haematol.2009.011429; Ambaglio I, 2013, HAEMATOLOGICA; Angelucci E, 2012, ASH ANN M, V120, P425; Angelucci E, 2000, NEW ENGL J MED, V343, P327, DOI 10.1056-NEJM200008033430503; Apotex, 2013, FERRIPROX DEF TABL O; Armand P, 2013, BONE MARROW TRANSPL, V48, P146, DOI 10.1038-bmt.2012.94; Baker E, 1998, BBA-GEN SUBJECTS, V1380, P21, DOI 10.1016-S0304-4165(97)00120-7; Breccia M, 2012, ANN HEMATOL, V91, P1345, DOI 10.1007-s00277-012-1481-7; Brissot P, 2012, BBA-GEN SUBJECTS, V1820, P403, DOI 10.1016-j.bbagen.2011.07.014; Cabantchik ZI, 2005, BEST PRACT RES CL HA, V18, P277, DOI 10.1016-j.beha.2004.10.003; Carpenter JP, 2011, CIRCULATION, V123, P1519, DOI 10.1161-CIRCULATIONAHA.110.007641; Cermak J, 2009, LEUKEMIA RES, V33, P1469, DOI 10.1016-j.leukres.2009.06.033; Chacko J, 2007, BRIT J HAEMATOL, V138, P587, DOI 10.1111-j.1365-2141.2007.06695.x; Cilloni D, 2011, ASH ANN M, V118, P611; de Swart L, 2011, ASH ANN M, V118, P2775; De Domenico I, 2009, BLOOD, V114, P4546, DOI 10.1182-blood-2009-05-224188; Delea TE, 2009, CURR MED RES OPIN, V25, P139, DOI 10.1185-03007990802565867 ; Di Tucci AA, 2008, HAEMATOL-HEMATOL J, V93, P1385, DOI 10.3324-haematol.12759; Fenaux P, 2013, BLOOD, V121, P4280, DOI 10.1182-blood-2013-02-453068; Forni GL, 2013, HAEMATOLOGICA, V98, P555, DOI 10.3324-haematol.2012.076240; Ganz T, 2006, AM J PHYSIOL-GASTR L, V290, pG199, DOI 10.1152-ajpgi.00412.2005.; Gardenghi S, 2010, J CLIN INVEST, V120, P4466, DOI 10.1172-JCI41717; Gattermann N, 2012, EUR J HAEMATOL, V88, P260, DOI 10.1111-j.1600-0609.2011.01726.x; Gattermann N, 2010, LEUKEMIA RES, V34, P1143, DOI 10.1016-j.leukres.2010.03.009; Gattermann N, 2012, HAEMATOL-HEMATOL J, V97, P1364, DOI 10.3324-haematol.2011.048546; Gattermann N, 2011, ANN HEMATOL, V90, P1, DOI 10.1007-s00277-010-1091-1; Ghoti H, 2011, BRIT J HAEMATOL, V153, P118, DOI 10.1111-j.1365-2141.2011.08587.x; Ginzburg Y, 2011, BLOOD, V118, P4321, DOI 10.1182-blood-2011-03-283614; Goldberg SL, 2010, J CLIN ONCOL, V28, P2847, DOI 10.1200-JCO.2009.25.2395; Gonzalez FA, 2005, MED CLIN-BARCELONA, V124, P645; Greenberg Peter L, 2006, J Natl Compr Canc Netw, V4, P91; Greenberg PL, 2010, LEUKEMIA RES, V34, P1560, DOI 10.1016-j.leukres.2010.06.013; Gunshin H, 1997, NATURE, V388, P482, DOI 10.1038-41343; Hankins JS, 2009, BLOOD, V113, P4853, DOI 10.1182-blood-2008-12-191643; Hartmann J, 2013, LEUKEMIA RES, V37, P327, DOI 10.1016-j.leukres.2012.11.005; HEBBEL RP, 1985, CLIN HAEMATOL, V14, P129; Hentze MW, 2010, CELL, V142, P24, DOI 10.1016-j.cell.2010.06.028; Hershko C, 1998, ANN NY ACAD SCI, V850, P191, DOI 10.1111-j.1749-6632.1998.tb10475.x; Ito K, 2006, NAT MED, V12, P446, DOI 10.1038-nm1388; Jensen PD, 1996, BRIT J HAEMATOL, V94, P288, DOI 10.1046-j.1365-2141.1996.d01-1795.x; Kersten MJ, 1996, ANN HEMATOL, V73, P247, DOI 10.1007-s002770050236; Konen E, 2007, AM J HEMATOL, V82, P1013, DOI 10.1002-ajh.20980; Leitch HA, 2008, CLIN LEUK, V2, P205, DOI 10.3816-CLK.2008.n.026; Leitch HA, 2012, LEUKEMIA RES, V36, P1380, DOI 10.1016-j.leukres.2012.08.001; Le Lan C, 2005, BLOOD, V105, P4527, DOI 10.1182-blood2004-09-3468; Li HH, 2010, NAT MED, V16, P177, DOI 10.1038-nm.2073; List AF, 2012, J CLIN ONCOL, V30, P2134, DOI 10.1200-JCO.2010.34.1222; Liuzzi JP, 2006, P NATL ACAD SCI USA, V103, P13612, DOI 10.1073-pnas.0606424103; Lyons RM, 2011, ASH ANN M, V118, P2800; Malcovati L, 2005, J CLIN ONCOL, V23, P7594, DOI 10.1200-JCO.2005.01.7038; Malcovati L, 2007, LEUKEMIA RES, V31, pS2, DOI 10.1016-S0145-2126(07)70459-9; Metzgeroth G, 2009, ANN HEMATOL, V88, P301, DOI 10.1007-s00277-008-0588-3; Mitchell M, 2013, EXPERT REV HEMATOL, V6, P397, DOI 10.1586-17474086.2013.814456; Nai A, 2012, BLOOD, V119, P5021, DOI 10.1182-blood-2012-01-401885; Neukirchen J, 2012, LEUKEMIA RES, V36, P1067, DOI 10.1016-j.leukres.2012.04.006; Nilsson-Ehle H, 2011, EUR J HAEMATOL, V87, P244, DOI 10.1111-j.1600-0609.2011.01654.x; Nolte F, 2013, ANN HEMATOL, V92, P191, DOI 10.1007-s00277-012-1594-z; Novartis, 2013, EXJADE DEF TABL OR; Novartis, 2013, DESF DEF MES INJ USP; Oliva EN, 2005, LEUKEMIA RES, V29, P1217, DOI 10.1016-j.leukres.2005.03.004; Olivieri NF, 1997, BLOOD, V89, P739; Oudit GY, 2003, NAT MED, V9, P1187, DOI 10.1038-nm920; Papaemmanuil E, 2011, NEW ENGL J MED, V365, P1384, DOI 10.1056-NEJMoa1103283; Porter J, 2008, EUR J HAEMATOL, V80, P168, DOI 10.1111-j.1600-0609.2007.00985.x; Rollison DE, 2008, BLOOD, V112, P45, DOI 10.1182-blood-2008-01-134858; Rose C, 2010, LEUKEMIA RES, V34, P864, DOI 10.1016-j.leukres.2009.12.004; Sallmyr A, 2008, CANCER LETT, V270, P1, DOI 10.1016-j.canlet.2008.03.036; Santini V, 2011, PLOS ONE, V6, DOI 10.1371-journal.pone.0023109; Santini V, 2010, LEUKEMIA RES, V34, P1576, DOI 10.1016-j.leukres.2010.01.018; Sanz G, 2008, ASH ANN M, V112, P640; Siah Chiang W, 2006, Clin Biochem Rev, V27, P5; Steensma DP, 2006, MAYO CLIN PROC, V81, P104; Taher Ali T, 2009, Hemoglobin, V33 Suppl 1, pS46, DOI 10.3109-03630260903346676; Takatoku M, 2007, EUR J HAEMATOL, V78, P487, DOI 10.1111-j.1600-0609.2007.00842.x; Tanno T, 2007, NAT MED, V13, P1096, DOI 10.1038-nm1629; Valent P, 2007, LEUKEMIA RES, V31, P727, DOI 10.1016-j.leukres.2006.11.009; Wood JC, 2011, HEMATOL-AM SOC HEMAT, P443, DOI 10.1182-asheducation-2011.1.443; WRIGHT TL, 1986, J BIOL CHEM, V261, P909; Zipperer E, 2013, ANN HEMATOL, V92, P1617, DOI 10.1007-s00277-013-1839-50
Guidelines for diagnosis and management of beta-thalassemia intermedia
No full textBeta-thalassemia intermedia (β-TI) is a genetic variant of beta-thalassemias with a clinical disorder whose severity falls between thalassemia minor and thalassemia major. Different genetic defects are involved in this disorder and, based on severity of disease, clinical complications like skeletal deformities and growth retardation, splenomegaly, extramedullary hematopoiesis, heart failure, and endocrine disorders may be present in untreated patients. Precise diagnosis and management are essential in these patients for prevention of later clinical complications. Diagnosis of TI is based on clinical and laboratory data. There are some treatment strategies like modulation of gamma-globulin chain production with hydroxyurea or other drugs, transfusion, splenectomy, and stem cell transplantation. Iron chelation therapy is also needed in many of these patients even if they are not transfused. The aim of this manuscript is to review the clinical manifestations, complications, genetic defects, and unmet treatments needs in TI.Aessopos A, 2007, ATHEROSCLEROSIS, V191, P427, DOI 10.1016-j.atherosclerosis.2006.04.015; Aessopos A, 2007, TRANSFUSION, V47, P792, DOI 10.1111-j.1537-2995.2007.01192.x; Amoozgar H, 2011, EUR J HAEMATOL, V85, P549; Atichartakarn V, 2003, INT J HEMATOL, V78, P139, DOI 10.1007-BF02983382; Cadili A, 2008, AM J MED, V121, P371, DOI 10.1016-j.amjmed.2008.02.014; CAMASCHELLA C, 1995, HAEMATOLOGICA, V80, P58; Camaschella C, 1997, AM J HEMATOL, V55, P83, DOI 10.1002-(SICI)1096-8652(199706)55:283::AID-AJH63.3.CO;2-M; Cappellini MD, 2005, SEMIN HEMATOL, V42, pS19, DOI 10.1053-j.seminhematol.2005.01.001; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; Derakhshan A, 2008, SAUDI J KIDNEY DIS T, V19, P206; De Sanctis V, 1998, J PEDIATR ENDOCR MET, V11, P965; Dixit A, 2005, ANN HEMATOL, V84, P441, DOI 10.1007-s00277-005-1026-4; Elalfy MS, 2013, EUR J HAEMATOL, V91, P522, DOI 10.1111-ejh.12182; El Rassi F, 2008, PEDIATR ANN, V37, P322; Galanello R, 1998, ANN NY ACAD SCI, V850, P325, DOI 10.1111-j.1749-6632.1998.tb10489.x; Gamberini MR1, 2004, PEDIAT ENDOCRINOL S2, P319; Gladwin MT, 2003, NAT MED, V9, P496, DOI 10.1038-nm0503-496; Gladwin MT, 2008, NEW ENGL J MED, V359, P2254, DOI 10.1056-NEJMra0804411; Haddad A, 2014, TURK J HEMATOL, V31, P5, DOI 10.4274-Tjh.2014.0032; Haghpanah S, 2014, HEMATOLOGY, V19, P187, DOI 10.1179-1607845413Y.0000000121; Harmatz P, 2008, HAEMATOL-HEMATOL J, V93, P1247, DOI 10.3324-haematol.12352; Karimi M, 2012, INT J HEMATOL, V95, P51, DOI 10.1007-s12185-011-0985-6; Karimi M, 2012, ANN HEMATOL, V91, P1833, DOI 10.1007-s00277-012-1527-x; Karimi M, 2009, EUR J HAEMATOL, V82, P213, DOI 10.1111-j.1600-0609.2008.01192.x; Karimi M, 2012, HEMATOLOGY, V17, P122, DOI 10.1179-102453312X13221316477778; Karimi M, 2010, EUR J HAEMATOL, V84, P52, DOI 10.1111-j.1600-0609.2009.01356.x; Karimi M, 2014, HEMATOLOGY; Karimi M, 2007, INT J LAB HEMATOL, V29, P321, DOI 10.1111-j.1365-2257.2006.00856.x; Karimi M, 2010, THROMB HAEMOSTASIS, V103, P989, DOI 10.1160-TH09-09-0661; Karimi M, 2011, EUR J INTERN MED, V22, P607, DOI 10.1016-j.ejim.2011.05.013; Karimi M, 2008, LANCET, V372, P1436, DOI 10.1016-S0140-6736(08)61590-1; Karimi M, 2008, AM J HEMATOL, V83, P77, DOI 10.1002-ajh.20938; Karimi M, 2005, J PEDIAT HEMATOL ONC, V27, P380, DOI 10.1097-01.mph.0000174386.13109.28; Karimi M, 2010, PEDIATR HEMAT ONCOL, V27, P205, DOI 10.3109-08880011003639952; Mancuso A, 2006, HEMOGLOBIN, V30, P119, DOI 10.1080-03630260500455565; Manfre L, 1999, AM J ROENTGENOL, V173, P1477; Matta BN, 2013, J EUR ACAD DERMATOL; Moorchung N, 2006, HAEMA, V9, P505; Musallam KM, 2011, EUR J HAEMATOL, V87, P73, DOI 10.1111-j.1600-0609.2011.01623.x; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Musallam KM, 2012, THROMB RES, V130, P695, DOI 10.1016-j.thromres.2012.07.013; Olivieri NF, 1999, NEW ENGL J MED, V341, P99, DOI 10.1056-NEJM199907083410207; Pakbaz Z, 2005, ANN NY ACAD SCI, V1054, P457, DOI 10.1196-annals.1345.059; Pierre T.G., 2005, BLOOD, V105, P855; Rachid H, 2010, EUR SPINE J, V19, P871; Rachmilewitz A, 2011, BLOOD, V118, P3479; Rachmilewitz EA, 1998, ANN NY ACAD SCI, V850, P129, DOI 10.1111-j.1749-6632.1998.tb10470.x; Pantalone GR, 2010, BRIT J HAEMATOL, V150, P245, DOI 10.1111-j.1365-2141.2010.08180.x; Rund D, 2005, NEW ENGL J MED, V353, P1135, DOI 10.1056-NEJMra050436; SPANOS T, 1990, VOX SANG, V58, P50; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; Taher A, 2006, BLOOD CELL MOL DIS, V37, P12, DOI 10.1016-j.bcmd.2006.04.005; Taher AT, 2013, ANN HEMATOL, V92, P1485, DOI 10.1007-s00277-013-1808-z; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2010, J THROMB HAEMOST, V8, P2152, DOI 10.1111-j.1538-7836.2010.03940.x; Taher AT, 2010, J THROMB HAEMOST, V8, P54, DOI 10.1111-j.1538-7836.2009.03651.x; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Taher AT, 2012, BLOOD REV, V26, pS24, DOI 10.1016-S0268-960X(12)70008-5; Thein SL, 2004, BRIT J HAEMATOL, V124, P264, DOI 10.1046-j.1365-2141.2003.04769.x; Voskaridou E, 2010, BRIT J HAEMATOL, V148, P332, DOI 10.1111-j.1365-2141.2009.07930.x; WEATHERALL D, 1995, MOL MED TODAY, V1, P15, DOI 10.1016-1357-4310(95)80014-X; Weatherall DJ, 2001, J HEMATOL S1, V86, P186; Weatherall DJ, 2001, NAT REV GENET, V2, P245, DOI 10.1038-35066048; Wood JC, 2005, BLOOD, V106, P1460, DOI 10.1182-blood-2004-10-39821
To the Editor
No full text[No abstract available]CABANNES R, 1965, NOUV REV FR HEMATOL, V5, P851; KHOURI FP, 1986, LEBANON MED J S2, V36, P69; Makhoul NJ, 2005, ANN HUM GENET, V69, P55, DOI 10.1046-j.1529-8817.2004.00138.x; Zahed L, 1997, HUM HERED, V47, P241, DOI 10.1159-000154419; Zahed L, 1997, PRENATAL DIAG, V17, P423, DOI 10.1002-(SICI)1097-0223(199705)17:5423::AID-PD683.0.CO;2-P0
Successful management of hydroxyurea-induced leg ulcers in essential thrombocythemia: Report of 3 cases [Hidroksiüre'nin Anlati[dotless]lmami[dotless]ş hikayesi: Bacak ülserli 3 olgunun tedavisi]
No full textEssential thrombocythemia is one of the myeloproliferative neoplasms with a plethora of thrombohemorrhagic complications. Hydroxyurea has been proven to be an effective treatment for this condition. However, it is not without side effects. We herein report 3 patients with essential thrombocythemia treated with hydroxyurea who developed refractory leg ulcers, and we outline their successful management. We also review the literature to shed light on the mechanism of this toxicity. Awareness of this important treatment complication is important to avoid the pitfall of futile invasive interventions.Demircay Z, 2002, INT J DERMATOL, V41, P872, DOI 10.1046-j.1365-4362.2002.01623.x; Kikuchi K, 2011, ACTA DERM-VENEREOL, V91, P373, DOI 10.2340-00015555-1048; Martorell-Calatayud A, 2009, Actas Dermosifiliogr, V100, P804; MIDDELHOFF G, 1992, ANN HEMATOL, V64, P207, DOI 10.1007-BF01738297; Najean Y, 1997, BLOOD, V90, P3370; PAPI M, 1993, J AM ACAD DERMATOL, V28, P485; Radaelli F, 1998, AM J HEMATOL, V58, P82, DOI 10.1002-(SICI)1096-8652(199805)58:182::AID-AJH163.0.CO;2-7; RENFRO L, 1991, J AM ACAD DERMATOL, V24, P143, DOI 10.1016-S0190-9622(08)80052-7; RICHARD M, 1989, J AM ACAD DERMATOL, V21, P797, DOI 10.1016-S0190-9622(89)80274-9; Ruzzon E, 2006, AGING CLIN EXP RES, V18, P187; Tefferi A, 2012, AM J HEMATOL, V87, P285, DOI 10.1002-ajh.23135; Tsuchiya S, 2010, J WOUND CARE, V19, P3610
Iron chelation therapy for patients with sickle cell disease and iron overload
No full textA 21-year-old male with sickle cell disease (SCD) presented with severe pallor. He had received a total of 100 red blood cell (RBC) units in his lifetime, had a mean serum ferritin level of 3133 ng-ml, and liver iron concentration (LIC) of 12 mg Fe-g dry weight (dw). He was started on subcutaneous deferoxamine (DFO) infusions at a dose of 56 mg-kg-d, five days a week (equivalent to 40 mg-kg-d, seven days a week) and continued to receive 8-10 RBC units-year as treatment for pain. During the first six months of chelation therapy, his serum ferritin levels fell by around 50percent of the pretreatment value, but then started to increase back up to the baseline values. The patient was noncompliant with DFO therapy. He experienced pain at the site of injection, could not sleep and was concerned about carrying a pump and not being accepted by his peers. He dropped out of college and abstained from all social activities. He was referred to a psychologist; however, this failed to improve compliance and he opted to stop DFO therapy altogether. © 2010 Wiley-Liss, Inc.Abetz L, 2006, HEALTH QUAL LIFE OUT, V4, DOI 10.1186-1477-7525-4-73; Adamkiewicz TV, 2009, BLOOD, V114, P4632, DOI 10.1182-blood-2009-02-203323; Adams RJ, 1998, NEW ENGL J MED, V339, P5, DOI 10.1056-NEJM199807023390102; Adams RJ, 2005, NEW ENGL J MED, V353, P2769; ALREFAIE FN, 1995, BRIT J HAEMATOL, V91, P224, DOI 10.1111-j.1365-2141.1995.tb05274.x; Andrews NC, 1999, NEW ENGL J MED, V341, P1986, DOI 10.1056-NEJM199912233412607; Angelucci E, 2008, HAEMATOL-HEMATOL J, V93, P741, DOI 10.3324-haematol.12413; Aydinok Y, 2005, PEDIATR INT, V47, P84, DOI 10.1111-j.1442-200x.2004.02009.x; Ballas SK, 2001, SEMIN HEMATOL, V38, P30, DOI 10.1016-S0037-1963(01)90058-7; Borgna-Pignatti C, 1998, ANN NY ACAD SCI, V850, P227, DOI 10.1111-j.1749-6632.1998.tb10479.x; BRITTENHAM GM, 1994, NEW ENGL J MED, V331, P567, DOI 10.1056-NEJM199409013310902; BRITTENHAM GM, 1993, AM J HEMATOL, V42, P81, DOI 10.1002-ajh.2830420116; Brown K, 2009, J PEDIAT HEMATOL ONC, V31, P309, DOI 10.1097-MPH.0b013e3181a1c143; Cabantchik ZI, 2005, BEST PRACT RES CL HA, V18, P277, DOI 10.1016-j.beha.2004.10.003; CANCADO R, 2009, HAEMATOLOGICA S2, V94; Cappellini MD, 2010, HAEMATOL-HEMATOL J, V95, P557, DOI 10.3324-haematol.2009.014696; Cappellini MD, 2006, BLOOD, V107, P3455, DOI 10.1182-blood-2005-08-3430; COHEN A, 1979, AM J HEMATOL, V7, P69, DOI 10.1002-ajh.2830070109; COHEN A, 1978, J PEDIATR, V92, P659, DOI 10.1016-S0022-3476(78)80317-5; COHEN AR, 1989, J PEDIATR, V115, P151, DOI 10.1016-S0022-3476(89)80353-1; COLLINS AF, 1994, BLOOD, V83, P2329; Darbari DS, 2006, AM J HEMATOL, V81, P858, DOI 10.1002-ajh.20685; *FERR, 2007, FERR PRESCR INF; FILLET G, 1989, BLOOD, V74, P844; Fung EB, 2006, BRIT J HAEMATOL, V135, P574, DOI 10.1111-j.1365-2141.2006.06332.x; Fung EB, 2007, AM J HEMATOL, V82, P255, DOI 10.1002-ajh.20809; Harmatz P, 2000, BLOOD, V96, P76; Hoffbrand AV, 1998, BLOOD, V91, P295; Hoffbrand AV, 2003, BLOOD, V102, P17, DOI 10.1182-blood-2002-06-1867; Inati A, 2010, BLOOD, V115, P2980, DOI 10.1182-blood-2009-09-243568; Inati A, 2009, EUR J HAEMATOL, V83, P565, DOI 10.1111-j.1600-0609.2009.01345.x; INATI A, 2010, INT J LAB HEMATOL, DOI DOI 10.1111-J.1751-553X.2010.01255.X; KALPATTHI R, 2008, BLOOD, V112; Karam LB, 2008, PEDIATR BLOOD CANCER, V50, P62, DOI 10.1002-pbc.21215; Kersten MJ, 1996, ANN HEMATOL, V73, P247, DOI 10.1007-s002770050236; Koren A, 2010, EUR J HAEMATOL, V84, P72, DOI 10.1111-j.1600-0609.2009.01342.x; Leonard MB, 1998, J PEDIATR, V132, P467, DOI 10.1016-S0022-3476(98)70022-8; Modell B, 2000, LANCET, V355, P2051, DOI 10.1016-S0140-6736(00)02357-6; Mokhtar GM, 2010, HEMOGLOBIN, V34, P78, DOI 10.3109-03630260903554621; National Institutes of Health National Heart Lung and Blood Institute. Global initiative for asthma, 2002, NIH PUBL; OLIVIERI NF, 1995, NEW ENGL J MED, V332, P918, DOI 10.1056-NEJM199504063321404; Olivieri NF, 2001, SEMIN HEMATOL, V38, P57, DOI 10.1053-shem.2001.20144; Olivieri NF, 1998, NEW ENGL J MED, V339, P417, DOI 10.1056-NEJM199808133390701; OLIVIERI NF, 1986, NEW ENGL J MED, V314, P869, DOI 10.1056-NEJM198604033141402; Pakbaz Z, 2007, PEDIATR BLOOD CANCER, V49, P329, DOI 10.1002-pbc.21275; PAKBAZ Z, 2004, BLOOD, V104; PORTER JB, 1987, ACTA HAEMATOL-BASEL, V78, P198; Porter JB, 2001, BRIT J HAEMATOL, V115, P239, DOI 10.1046-j.1365-2141.2001.03195.x; Porter JB, 2008, BLOOD, V112; Porter J, 2008, EUR J HAEMATOL, V80, P168, DOI 10.1111-j.1600-0609.2007.00985.x; Schmid M, 2009, BLOOD, V114; Sickle Cell Society, 2008, STAND CLIN CAR AD SI; SILLIMAN CC, 1993, J LAB CLIN MED, V122, P48; Stuart MJ, 2004, LANCET, V364, P1343, DOI 10.1016-S0140-6736(04)17192-4; Treadwell MJ, 2005, PEDIATR BLOOD CANCER, V44, P500, DOI 10.1002-pbc.20290; Verduzco LA, 2009, BLOOD, V114, P5117, DOI 10.1182-blood-2009-05-220921; Vichinsky E, 2007, BRIT J HAEMATOL, V136, P501, DOI 10.1111-j.1365-2141.2006.06455.x; VICHINSKY E, 2008, BLOOD, V112; Vichinsky E, 2005, AM J HEMATOL, V80, P70, DOI 10.1002-ajh.20402; Vichinsky E, 2008, AM J HEMATOL, V83, P398, DOI 10.1002-ajh.21119; Voskaridou E, 2005, ANN HEMATOL, V84, P434, DOI 10.1007-s00277-005-1015-7; Voskaridou E, 2009, HAEMATOLOGICA S2, V94; Voskaridou E, 2007, HAEMATOL-HEMATOL J, V92, P738, DOI 10.3324-haematol.11136; Wahl S, 2009, CURR OPIN PEDIATR, V21, P15, DOI 10.1097-MOP.0b013e328321882e; Walter PB, 2006, BRIT J HAEMATOL, V135, P254, DOI 10.1111-j.1365-2141.2006.06277.x; Wonke B, 1998, BRIT J HAEMATOL, V103, P361; Wood JC, 2004, BLOOD, V103, P1934, DOI 10.1182-blood-2003-06-1919; ZURLO MG, 1989, LANCET, V2, P2787
Brain positron emission tomography in splenectomized adults with β-thalassemia intermedia: Uncovering yet another covert abnormality
No full textCovert brain infarction is an emerging concern in patients with β-thalassemia intermedia (TI). We have recently observed a high prevalence (60percent) of silent brain infarction on brain magnetic resonance imaging (MRI) in 30 splenectomized adults with TI. In this work, we further evaluate cerebral involvement in the same 30 patients using fluorodeoxyglucose positron emission tomography-computed tomography (PET-CT) scanning. The median age was 32 years (range, 18-54 years) with a male to female ratio of 13:17. Nineteen patients (63.3percent) had evidence of decreased neuronal function on PET-CT. Involvement was mostly left sided, multiple, and most commonly in the temporal and parietal lobes. Elevated liver iron concentration, beyond 15 mg Fe-g dry weight, characterized patients with decreased neuronal function. The concordance rate between brain MRI and PET-CT for the detection of brain abnormality was only 36.7percent (Kappa 0.056, P = 0.757), highlighting that both modalities reveal different types of brain pathology. Decreased neuronal function is a common finding in patients with TI and is associated with iron overload. Moreover, the addition of PET-CT to MRI identifies a greater proportion of TI patients with silent neuroimaging abnormalities. © 2011 Springer-Verlag.Afifi AA, 2004, COMPUTER AIDED MULTI, P489; Arkuszewski M, 2010, ADV MED SCI-POLAND, V55, P115, DOI 10.2478-v10039-010-0045-0; Armstrong FD, 1996, PEDIATRICS, V97, P864; Atichartakarn V, 2002, BRIT J HAEMATOL, V118, P893, DOI 10.1046-j.1365-2141.2002.03711.x; Bernaudin F, 2000, J CHILD NEUROL, V15, P333, DOI 10.1177-088307380001500510; CAMASCHELLA C, 1995, HAEMATOLOGICA, V80, P58; Cappellini MD, 2010, ANN NY ACAD SCI, V1202, P231, DOI 10.1111-j.1749-6632.2010.05548.x; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; DeReuck J, 1997, ACTA NEUROL BELG, V97, P168; GOOD PF, 1992, ANN NEUROL, V31, P286, DOI 10.1002-ana.410310310; Gross H, 1996, J NEUROPSYCH CLIN N, V8, P324; HEROLD S, 1986, STROKE, V17, P692; Hoffman JM, 2000, J NUCL MED, V41, P1920; KUGLER S, 1993, ARCH NEUROL-CHICAGO, V50, P629; Labbe C, 1996, ALZ DIS ASSOC DIS, V10, P141, DOI 10.1097-00002093-199601030-00005; Li XP, 2011, J NEURAL TRANSM, V118, P473, DOI 10.1007-s00702-010-0518-0; Manfre L, 1999, AM J ROENTGENOL, V173, P1477; MEHTA RC, 1992, CURR OPIN RADIOL, V4, P95; Miller ST, 2001, J PEDIATR-US, V139, P385, DOI 10.1067-mpd.2001.117580; Origa R, 2008, HAEMATOL-HEMATOL J, V93, P1095, DOI 10.3324-haematol.12484; Pegelow CH, 2002, BLOOD, V99, P3014, DOI 10.1182-blood.V99.8.3014; Pegelow CH, 2001, ARCH NEUROL-CHICAGO, V58, P2017, DOI 10.1001-archneur.58.12.2017; Powars DR, 1999, BLOOD, V93, P71; Reed W, 1999, AM J HEMATOL, V60, P268, DOI 10.1002-(SICI)1096-8652(199904)60:4268::AID-AJH33.0.CO;2-C; RODGERS GP, 1988, ARCH NEUROL-CHICAGO, V45, P78; Roghi A, 2010, ANN HEMATOL, V89, P585, DOI 10.1007-s00277-009-0879-3; Smith MA, 1997, P NATL ACAD SCI USA, V94, P9866, DOI 10.1073-pnas.94.18.9866; Steinberg MH, 2009, DISORDERS OF HEMOGLOBIN: GENETICS, PATHOPHYSIOLOGY, AND CLINICAL MANAGEMENT, 2ND EDITION, P1, DOI 10.1017-CBO9780511596582; St Pierre TG, 2005, BLOOD, V105, P855, DOI 10.1182-blood-2004-01-0177; Switzer JA, 2006, LANCET NEUROL, V5, P501, DOI 10.1016-S1474-4422(06)70469-0; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; Taher A, 2009, BRIT J HAEMATOL, V147, P634, DOI 10.1111-j.1365-2141.2009.07848.x; Taher AT, 2010, AM J HEMATOL, V85, P288, DOI 10.1002-ajh.21626; Taher AT, 2010, BRIT J HAEMATOL, V150, P486, DOI 10.1111-j.1365-2141.2010.08220.x; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2010, J THROMB HAEMOST, V8, P2152, DOI 10.1111-j.1538-7836.2010.03940.x; Taher AT, 2010, J THROMB HAEMOST, V8, P54, DOI 10.1111-j.1538-7836.2009.03651.x; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Verduzco LA, 2009, BLOOD, V114, P5117, DOI 10.1182-blood-2009-05-220921; Vichinsky EP, 2010, JAMA-J AM MED ASSOC, V303, P1823, DOI 10.1001-jama.2010.562; Wang W, 2001, J PEDIATR-US, V139, P391, DOI 10.1067-mpd.2001.1169359121
β-Thalassemia intermedia: A bird'S-eye view [β-Talasemi i̇ntermedya: Kuşbaki[dotless]şi[dotless]]
No full textBeta-thalassemia is due to a defect in the synthesis of the beta-globin chains, leading to alpha-beta imbalance, ineffective erythropoiesis, and chronic anemia. The spectrum of thalassemias is wide, with one end comprising thalassemia minor, which consists of a mild hypochromic microcytic anemia with no obvious clinical manifestations, while on the other end is thalassemia major, characterized by patients who present in their first years of life with profound anemia and regular transfusion requirements for survival. Along the spectrum lies thalassemia intermedia, a term developed to describe patients with manifestations that are neither mild enough nor severe enough to be classified in the spectrum's extremes. Over the past decade, our understanding of β-thalassemia intermedia has increased tremendously with regards to molecular information as well as pathophysiology. It is now clear that β-thalassemia intermedia has a clinical presentation as well as complications associated with the disease that are different from those of β-thalassemia major. This review is designed to tackle issues related to β-thalassemia intermedia from the basic definition of the disease to paramedical issues, namely the quality of life in these patients. Genetics and pathophysiology are revisited, as well as the complications specific to this disease. These complications include effects on several organ systems, including the cardiovascular, hepatic, endocrine, renal, brain, and skeletal systems. Extramedullary hematopoiesis is also discussed in this article. Risk factors are highlighted and cutoffs are identified to minimize morbidities in β-thalassemia intermedia. Several treatment modalities are considered by shining a light on the pros and cons of each modality, as well as the role of special pharmacological agents in the progress of the disease and its morbidities. Finally, health-related quality of life is discussed in these patients with a direct comparison to the more severe β-thalassemia major.Aessopos A, 2007, ATHEROSCLEROSIS, V191, P427, DOI 10.1016-j.atherosclerosis.2006.04.015; Aessopos A, 2006, ANN THORAC SURG, V81, P2037, DOI 10.1016-j.athoracsur.2006.01.026; Atichartakarn V, 2003, INT J HEMATOL, V78, P139, DOI 10.1007-BF02983382; Borgna-Pignatti C, 2010, ANN NY ACAD SCI, V1202, P214, DOI 10.1111-j.1749-6632.2010.05550.x; Borgna-Pignatti C, 2007, BRIT J HAEMATOL, V138, P291, DOI 10.1111-j.1365-2141.2007.06654.x; Brandt HRC, 2009, BRIT J DERMATOL, V160, P202, DOI 10.1111-j.1365-2133.2008.08894.x; Brannan D, 1927, B JOHNS HOPKINS HOSP, V41, P104; Brenner BM, 1996, KIDNEY INT, V49, P1774, DOI 10.1038-ki.1996.265; BREZIS M, 1995, NEW ENGL J MED, V332, P647; CAMASCHELLA C, 1995, HAEMATOLOGICA, V80, P58; CAMASCHELLA C, 1995, AM J HEMATOL, V48, P82; Cao A, 2013, CSH PERSPECT MED, V3, DOI 10.1101-cshperspect.a011775; Cappellini MD, 2009, DISORDERS ERYTHROPOI; Cappellini MD, 2005, SEMIN HEMATOL S1, V42, P19; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; Cappellini MD, 2001, HAEMATOLOGICA S1, V86, P194; Cappellini MD, 2006, BLOOD, V107, P3455, DOI 10.1182-blood-2005-08-3430; Chuang CK, 1998, J FORMOS MED ASSOC, V97, P431; Cooley TB, 1927, AM J DIS CHILD, V34, P347; Crary SE, 2009, BLOOD, V114, P2861, DOI 10.1182-blood-2009-04-210112; DAVIS LE, 1991, AM J PHYSIOL, V261, pR1542; Derchi G, 2014, CIRCULATION, V129, P338, DOI 10.1161-CIRCULATIONAHA.113.002124; Dixit A, 2005, ANN HEMATOL, V84, P441, DOI 10.1007-s00277-005-1026-4; DORE F, 1993, AM J HEMATOL, V44, P148, DOI 10.1002-ajh.2830440216; Dore F, 1992, Ann Ital Med Int, V7, P137; Galanello R, 1998, ANN NY ACAD SCI, V850, P325, DOI 10.1111-j.1749-6632.1998.tb10489.x; GALANELLO R, 1994, BLOOD, V83, P561; GIMMON Z, 1982, PLAST RECONSTR SURG, V69, P320, DOI 10.1097-00006534-198202000-00023; Gladwin MT, 2003, NAT MED, V9, P496, DOI 10.1038-nm0503-496; Gladwin MT, 2008, NEW ENGL J MED, V359, P2254, DOI 10.1056-NEJMra0804411; Habib A, 2008, HAEMATOL-HEMATOL J, V93, P941, DOI 10.3324-haematol.12460; Haidar R, 2010, EUR SPINE J, V19, P871, DOI 10.1007-s00586-010-1357-2; HALLIWELL B, 1990, METHOD ENZYMOL, V186, P1; Hershko C, 2010, ANN NY ACAD SCI, V1202, P1, DOI 10.1111-j.1749-6632.2010.05544.x; Heyman SN, 2008, AM J NEPHROL, V28, P998, DOI 10.1159-000146075; HMIDA S, 1994, NOUV REV FR HEMATOL, V36, P363; INGRAM VM, 1959, NATURE, V184, P1903, DOI 10.1038-1841903a0; Karimi M, 2005, J PEDIAT HEMATOL ONC, V27, P380, DOI 10.1097-01.mph.0000174386.13109.28; Khan S, 1999, LAB INVEST, V79, P1089; Khoury B, 2012, INT J PSYCHIAT MED, V44, P291, DOI 10.2190-PM.44.4.a; KUMAR A, 1995, SEMIN ROENTGENOL, V30, P99, DOI 10.1016-S0037-198X(05)80027-6; LAFFERTY HM, 1991, AM J KIDNEY DIS, V17, P2; Levin C, 2011, ISR MED ASSOC J, V13, P316; Li XP, 2011, J NEURAL TRANSM, V118, P473, DOI 10.1007-s00702-010-0518-0; Logothetis J, 1972, NEUROLOGY, V22, P249; Maakaron JE, 2013, ANN HEPATOL, V12, P142; Maakaron Joseph E, 2013, J Med Liban, V61, P175; Manfre L, 1999, AM J ROENTGENOL, V173, P1477; MASTRANGELO F, 1975, NEPHRON, V14, P229, DOI 10.1159-000180452; Musallam KM, 2012, BLOOD CELL MOL DIS, V49, P136, DOI 10.1016-j.bcmd.2012.06.001; Musallam KM, 2011, EUR J HAEMATOL, V87, P73, DOI 10.1111-j.1600-0609.2011.01623.x; Musallam KM, 2011, BLOOD CELL MOL DIS, V47, P232, DOI 10.1016-j.bcmd.2011.07.005; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Musallam KM, 2012, CSH PERSPECT MED, V2, DOI 10.1101-cshperspect.a013482; Musallam KM, 2012, BLOOD, V120; Musallam KM, 2012, THROMB RES, V130, P695, DOI 10.1016-j.thromres.2012.07.013; Nangaku M, 2006, J AM SOC NEPHROL, V17, P17, DOI 10.1681-ASN.2005070757; Neufeld EJ, 2012, BLOOD, V119, P3263, DOI 10.1182-blood-2011-10-386268; Norman JT, 2000, KIDNEY INT, V58, P2351, DOI 10.1046-j.1523-1755.2000.00419.x; Norman JT, 1999, EXP NEPHROL, V7, P463; Olivieri NF, 1999, NEW ENGL J MED, V341, P99, DOI 10.1056-NEJM199907083410207; Olivieri NF, 1996, SEMIN HEMATOL, V33, P24; Origa R, 2008, HAEMATOL-HEMATOL J, V93, P1095, DOI 10.3324-haematol.12484; Origa R, 2007, HAEMATOL-HEMATOL J, V92, P583, DOI 10.3324-haematol.10842; Pakbaz Z, 2005, ANN NY ACAD SCI, V1054, P457, DOI 10.1196-annals.1345.059; Panigrahi I, 2005, HEMATOLOGY, V10, P61, DOI 10.1080-10245330400020439; PARSA K, 1995, J NEUROSURG, V82, P657, DOI 10.3171-jns.1995.82.4.0657; Phrommintikul A, 2006, HEART, V92, P1467, DOI 10.1136-hrt.2005.079970; PIPPARD MJ, 1982, BLOOD, V60, P288; Ponticelli C, 2010, BLOOD REV, V24, P239, DOI 10.1016-j.blre.2010.08.004; Pootrakul P, 2003, BRIT J HAEMATOL, V122, P305, DOI 10.1046-j.1365-2141.2003.04412.x; PRABHAKAR S, 1980, SURG NEUROL, V13, P351; Quinn CT, 2011, BRIT J HAEMATOL, V153, P111, DOI 10.1111-j.1365-2141.2010.08477.x; Ricchi P, 2012, BLOOD CELL MOL DIS, V49, P133, DOI 10.1016-j.bcmd.2012.05.012; Richter E, 1993, Aktuelle Radiol, V3, P320; Rienhoff HY, 2011, HAEMATOL-HEMATOL J, V96, P521, DOI 10.3324-haematol.2010.034405; ROSS P, 1969, AMER J ROENTGENOL RA, V106, P604; Rund D, 2005, NEW ENGL J MED, V353, P1135, DOI 10.1056-NEJMra050436; Salehi SA, 2004, SPINAL CORD, V42, P117, DOI 10.1038-sj.sc.3101544; SHIN KH, 1994, ACTA HAEMATOL-BASEL, V91, P154; SONAKUL D, 1980, Southeast Asian Journal of Tropical Medicine and Public Health, V11, P516; SPANOS T, 1990, VOX SANG, V58, P50; St Pierre TG, 2005, BLOOD, V105, P855, DOI 10.1182-blood-2004-01-0177; STURGEON P, 1955, BRIT J HAEMATOL, V1, P264, DOI 10.1111-j.1365-2141.1955.tb05509.x; Sumboonnanonda A, 2003, PEDIATR NEPHROL, V18, P257, DOI 10.1007-s00467-003-1067-7; Sumiyoshi A, 1992, Southeast Asian J Trop Med Public Health, V23 Suppl 2, P29; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Taher A, 2006, BLOOD CELL MOL DIS, V37, P12; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; Taher A, 2009, BRIT J HAEMATOL, V147, P634, DOI 10.1111-j.1365-2141.2009.07848.x; Taher AT, 2008, BLOOD REV, V22, P283, DOI 10.1016-j.blre.2008.04.001; Taher AT, 2013, ANN HEMATOL, V92, P1485, DOI 10.1007-s00277-013-1808-z; Taher AT, 2010, AM J HEMATOL, V85, P288, DOI 10.1002-ajh.21626; Taher AT, 2010, BRIT J HAEMATOL, V150, P486, DOI 10.1111-j.1365-2141.2010.08220.x; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2010, J THROMB HAEMOST, V8, P2152, DOI 10.1111-j.1538-7836.2010.03940.x; Taher AT, 2010, J THROMB HAEMOST, V8, P54, DOI 10.1111-j.1538-7836.2009.03651.x; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Tanaka T, 2003, KIDNEY INT, V64, P2020, DOI 10.1046-j.1523-1755.2003.00301.x; Thalassemia International Federation, 2004, GUID CLIN MAN THAL; Treadwell MJ, 2001, SEMIN HEMATOL, V38, P77, DOI 10.1053-shem.2001.20148; Vichinsky E, 2007, BRIT J HAEMATOL, V136, P501, DOI 10.1111-j.1365-2141.2006.06455.x; WEATHERALL DJ, 1980, TEX REP BIOL MED, V40, P323; WEATHERA.DJ, 1965, NATURE, V208, P1061, DOI 10.1038-2081061a0; Weatherall DJ, 2011, TRANSFUSION MED, V21, P218, DOI 10.1111-j.1365-3148.2011.01090.x; Weatherall DJ, 2004, NAT REV GENET, V5, P625, DOI 10.1038-nrg1406; Whipple GH, 1936, J PEDIATR, V9, P279, DOI 10.1016-S0022-3476(36)80021-3; Wood JC, 2005, BLOOD, V106, P1460, DOI 10.1182-blood-2004-10-3982; Zeisberg M, 2000, J NEPHROL S3, V13, P11111
Absence of cardiac siderosis despite hepatic iron overload in Italian patients with thalassemia intermedia: An MRI T2* study
No full textCardiac involvement in patients with thalassemia intermedia (TI) is characterized by a high-output state and pulmonary hypertension, with systolic left ventricle function usually being preserved. Myocardial iron overload in patients with TI has not been extensively studied. We conducted a cross-sectional study of 49 Italian patients with TI. Patient charts were reviewed and data collected for transfusion and iron chelation history, status of the spleen, and comorbid illnesses or infections. Blood samples were obtained for assessment of hemoglobin, serum ferritin, and liver enzyme levels. Doppler echocardiography was done for all patients. Cardiac and hepatic iron levels were measured by magnetic resonance imaging T2*. The mean age was 40.5∈±∈8.3 years, with a male to female ratio of 29:20. A total of 34 (69.4percent) patients were splenectomized, and four patients had evidence of hepatitis C infection. Around 45percent of patients were transfusion naïve while the rest received infrequent (47percent) or regular (8percent) transfusions. A total of 31 (63.3percent) patients were maintained on iron chelation therapy. None of the patients had evidence of heart failure. Mean serum ferritin and liver iron concentration were 1,060.2 ng-ml and 8.2 mg Fe per gram dry weight, respectively. None of the patients had evidence of cardiac iron overload (mean cardiac T2*∈=∈38.7∈±∈11.0 ms). There were no statistically significant correlation between cardiac T2* values and liver iron concentration, serum ferritin, or any patient, disease, or treatment-related parameters. Patients with TI show absence of cardiac iron overload even if hepatic iron accumulation is significant. © 2009 Springer-Verlag.Aessopos A, 2005, CHEST, V127, P1523, DOI 10.1378-chest.127.5.1523; Aessopos A, 2007, HAEMATOL-HEMATOL J, V92, P658, DOI 10.3324-haematol.10915; Anderson LJ, 2001, EUR HEART J, V22, P2171, DOI 10.1053-euhj.2001.2822; CAMASCHELLA C, 1995, HAEMATOLOGICA, V80, P58; CAPPELLINI MD, 2009, ESH CLUB GLOBULE ROU, pCH12; Gardenghi S, 2007, BLOOD, V109, P5027, DOI 10.1182-blood-2006-09-048868; Inati A, 2009, EUR J HAEMATOL, V83, P565, DOI 10.1111-j.1600-0609.2009.01345.x; Jensen PD, 2003, BLOOD, V101, P4632, DOI 10.1182-blood-2002-09-2754; KLEBER FX, 1992, BRIT HEART J, V67, P289; Noetzli LJ, 2008, BLOOD, V112, P2973, DOI 10.1182-blood-2008-04-148767; Origa R, 2008, HAEMATOL-HEMATOL J, V93, P1095, DOI 10.3324-haematol.12484; Origa R, 2007, HAEMATOL-HEMATOL J, V92, P583, DOI 10.3324-haematol.10842; Pakbaz Z, 2007, PEDIATR BLOOD CANCER, V49, P329, DOI 10.1002-pbc.21275; PEPE A, 2006, J AM COLL CARDIOL, V47, P136; Raman SV, 2006, HAEMATOL-HEMATOL J, V91, P1329; Silvestri L, 2008, BLOOD, V111, P924, DOI 10.1182-blood-2007-07-100677; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Tanner MA, 2006, J CARDIOV MAGN RESON, V8, P543, DOI 10.1080-10976640600698155; Tanno T, 2007, NAT MED, V13, P1096, DOI 10.1038-nm1629; Voskaridou E, 2004, BRIT J HAEMATOL, V126, P736, DOI 10.1111-j.1365-2141.2004.05104.x; Wood JC, 2005, BLOOD, V106, P1460, DOI 10.1182-blood-2004-10-3982; Wood JC, 2004, BLOOD, V103, P1934, DOI 10.1182-blood-2003-06-1919; Wood JC, 2007, CURR OPIN HEMATOL, V14, P183, DOI 10.1097-MOH.0b013e3280d2b76b20202
- …
