Turkish Journal of Hematology Volume: 31 - Issue: 2

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Turk J Hematol 2014;31:168-174 Tiftik NR, et al: Rho/ROCK and Erythrocytes 15. Liao JK, Seto M, Noma K. Rho kinase (ROCK) inhibitors. J Cardiovasc Pharmacol 2007;50:17-24. 16. Takai Y, Sasaki T, Matazaki T. Small GTP-binding proteins. Physiol Rev 2001;81:153-208. 17. Bao W, Hu E, Tao L, Boyce R, Mirabile R, Thudium DT, Ma XL, Willette RN, Yue TL. Inhibition of Rho-kinase protects the heart against ischemia/reperfusion injury. Cardiovasc Res 2004;61:548-558. 18. Büyükafşar K, Arıkan O, Ark M, Seçilmiş A, Ün İ, Şingirik E. Rho-kinase expression and its contribution to the control of perfusion pressure in the isolated rat mesenteric vascular bed. Eur J Pharmacol 2004;485:263-268. 19. Kandabashi T, Shimokawa H, Miyata K, Kunihiro I, Kawano Y, Fukata Y, Higo T, Egashira K, Takahashi S, Kaibuchi K, Takeshita A. Inhibition of myosin phosphatase by upregulated Rho-kinase plays a key role for coronary artery spasm in a porcine model with interleukin-1β. Circulation 2000;101:1319-1323. 20. 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Case Report DOI: 10.4274/Tjh.2012.0151 Gelatinous Marrow Transformation: A Series of 11 Cases from a Tertiary Care Centre in South India Jelatinöz Kemik İliği Transformasyonu: Güney Hindistan’da Üçüncü Basamak Bir Merkezden 11 Olguluk Bir Seri Sreeya Das, Pritinanda Mishra, Rakhee Kar, Debdatta Basu Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Department of Pathology, Puducherry, India Abstract: Gelatinous marrow transformation (GMT) or serous atrophy of bone marrow (BM) is a rare disease characterised by focal marrow hypoplasia, fat atrophy, and accumulation of extracellular mucopolysaccharides abundant in hyaluronic acid. This study reviews 11 cases of GMT from South India. Clinical and haematological parameters, BM aspirate, and biopsies of all patients diagnosed with GMT over a period of 7 years were studied. GMT was diagnosed in BM biopsy based on characteristic morphological appearance and was confirmed by alcian blue positive staining pattern at pH levels of 2.5 and 0.5. Eleven patients were diagnosed with GMT. All were males within the age range of 15 to 50 years. The underlying clinical diagnosis was human immunodeficiency virus positivity in 5 cases, 2 with coexistent disseminated tuberculosis, 1 with cryptococcal meningitis, and 1 with oral candidiasis; disseminated tuberculosis in 1 case; pyrexia of unknown origin in 2 cases; Hodgkin’s lymphoma in 1 case; acute lymphoblastic lymphoma with maintenance chemotherapy in 1 case; and alcoholic pancreatitis in 1 case. BM aspirates showed gelatinous metachromatic seromucinous material in 3 cases. BM biopsies were hypocellular in 7 and normocellular in 4 cases and showed focal GMT in 5 and diffuse GMT in 6 cases. Reactive changes were seen in 4 cases and haemophagocytosis in addition to GMT in 1 case. GMT is a relatively uncommon condition and an indicator of severe illness. It should be differentiated from myelonecrosis, amyloidosis, and marrow oedema. A high index of suspicion is required to diagnose this condition. Key Words: Gelatinous transformation, Bone marrow Özet: Jelatinöz kemik iliği transformasyonu (JKİT) veya kemik iliğinin (Kİ) seröz atrofisi fokal ilik hipoplazisi, yağ atrofisi ve hyalürinik asitten zengin ekstrasellüler mukopolisakkarit birikimi ile karakterize nadir bir hastalıktır. Bu çalışma Güney Hindistan’dan 11 JKİT olgusunu gözden geçirmektedir. Yedi yıllık periyot boyunca JKİT tanısı alan tüm hastaların klinik ve hematolojik parametreler, Kİ aspirasyon ve biyopsileri incelendi. JKİT tanısı Kİ biyopsisinde karakteristik morfolojik görünüm temel alınarak konuldu, 2,5 ve 0,5 pH düzeyinde pozitif alcian mavi ile boyanarak doğrulandı. On iki hasta JKİT tanısı aldı. Tümü 15-50 yaş aralığında olan erkeklerdi. Altta yatan klinik tanı; 5 olguda insan bağışık yetmezlik virüs pozitifliği olup iki olguda eşlik eden dissemine tüberküloz, bir olguda kriptokokal menenjit ve bir olguda oral kandidiyazis saptandı. Bir olgu dissemine tüberküloz; iki olgu sebebi bilinmeyen yüksek ateş; bir olgu Hodgkin lenfoma; bir olgu idame tedavisi alan akut lenfoblastik lenfoma; ve bir olguda alkolik pankreatit idi. Kİ aspiratları 3 olguda jelatinöz metakromatik seromüsinöz yapı gösterdi. Kİ biyopsileri 7 olguda hiposellüler ve 4 olguda normosellüler idi. Beş olguda fokal JKİT, 6 olguda diffüz JKİT saptandı. Dört olguda reaktif değişiklikler ve bir olguda JKİT’ye ek olarak hemofagositoz izlendi. JKİT nispeten nadir bir durumdur ve ciddi bir hastalığın habercisidir. Miyelonekroz, amiloidoz, ve ilik ödem ile ayırıcı tanısı yapılmalıdır. Tanı için söz konusu durumdan şüphelenilmiş olması gerekir. Anahtar Sözcükler: Jelatinöz transformasyon, Kemik iliği Address for Correspondence: Rakhee Kar, M.D., Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Department of Pathology, Puducherry, India Phone: 91-9487896560 E-mail: rakhee_kar@rediffmail.com Received/Geliş tarihi : October 11, 2012 Accepted/Kabul tarihi : May 6, 2013 175
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