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Dergiler / Marmara Medical Journal / 2016 / Cilt: 29 - Sayı: 2

Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence

Rafet EREN
Tayfur TOPTAŞ
İşık Kaygusuz ATAGÜNDÜZ
Tülin Fıratlı TUĞLULAR
Sayfa
114–116
Yayın Tarihi
2016-04-01
DOI
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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

Catastrophic thromboembolic events that could not be controlled with aggressive anticoagulation in a profoundly thrombocytopenic patient without overt disseminated intravascular coagulation (DIC) may suggest co-existent PNH.Keywords: Leukemia, Promyelocytic, Acute, Hemoglobinuria, Paroxysmal

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Amaç & Hipotez:A previously healthy 31-year-old female presenting with dyspnea, iron deficiency anemia, pancytopenia, splenomegaly, and abnormal coagulation tests was admitted to the hospital.
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Temel Bulgular & Veri:Hematology consultation additionally revealed that 71% of cells were indicative of acute promyelocytic leukemia (APL) and the patient tested positive for a t(15;17) translocation, confirming APL.
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Sonuç & Katkı:Catastrophic thromboembolic events that could not be controlled with aggressive anticoagulation in a profoundly thrombocytopenic patient without overt disseminated intravascular coagulation (DIC) may suggest co-existent PNH.Keywords: Leukemia, Promyelocytic, Acute, Hemoglobinuria, Paroxysmal
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Lösemi · Promyelositik · Akut

Özet

A previously healthy 31-year-old female presenting with dyspnea, iron deficiency anemia, pancytopenia, splenomegaly, and abnormal coagulation tests was admitted to the hospital. Hematology consultation additionally revealed that 71% of cells were indicative of acute promyelocytic leukemia (APL) and the patient tested positive for a t(15;17) translocation, confirming APL. All-trans retinoic acid (ATRA) therapy was initiated immediately, but the patient exhibited severe dyspnea. This subsequently resulted in circulatory and respiratory arrest, followed by death. Just after death, fluorescein-labelled proaerolysin (FLAER) revealed a paroxysmal nocturnal hemoglobinuria (PNH) monocyteclone of 82%, confirming the diagnosis of PNH. Leukemia can be derived from non-PNH clones in PNH patients. Catastrophic thromboembolic events that could not be controlled with aggressive anticoagulation in a profoundly thrombocytopenic patient without overt disseminated intravascular coagulation (DIC) may suggest co-existent PNH.Keywords: Leukemia, Promyelocytic, Acute, Hemoglobinuria, Paroxysmal

Abstract

A previously healthy 31-year-old female presenting with dyspnea, iron deficiency anemia, pancytopenia, splenomegaly, and abnormal coagulation tests was admitted to the hospital. Hematology consultation additionally revealed that 71% of cells were indicative of acute promyelocytic leukemia (APL) and the patient tested positive for a t(15;17) translocation, confirming APL. All-trans retinoic acid (ATRA) therapy was initiated immediately, but the patient exhibited severe dyspnea. This subsequently resulted in circulatory and respiratory arrest, followed by death. Just after death, fluorescein-labelled proaerolysin (FLAER) revealed a paroxysmal nocturnal hemoglobinuria (PNH) monocyteclone of 82%, confirming the diagnosis of PNH. Leukemia can be derived from non-PNH clones in PNH patients. Catastrophic thromboembolic events that could not be controlled with aggressive anticoagulation in a profoundly thrombocytopenic patient without overt disseminated intravascular coagulation (DIC) may suggest co-existent PNH.Keywords: Leukemia, Promyelocytic, Acute, Hemoglobinuria, Paroxysmal

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Bu Makaleye Atıf Yap

EREN, R., TOPTAŞ, T., ATAGÜNDÜZ, İ. K., TUĞLULAR, T. F. (2016). Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence. Marmara Medical Journal, Cilt: 29 - Sayı: 2, 114–116. https://ajindex.com/articles/688350

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Kaynakça (4)

  1. Parker CJ. Paroxysmal nocturnal hemoglobinuria. Curr Opin Hematol 2012;19:141-8. doi: 10.1097/MOH.0b013e328351c348 Harris JW, Koscick R, Lazarus HM, Eshleman JR, Medof ME. Leukemia arising out of paroxysmal nocturnal hemoglobinuria. Leuk Lymphoma 1999;32:401-26.
  2. Levi M, Toh CH, Thachil J, Watson HG. Guidelines for the diagnosis and management of disseminated intravascular coagulation. Br J Haematol 2009;145:24-33. doi: 10.1111/j.13652141.2009.07600.x. Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, Hillmen P, Luzzatto L, Young N, Kinoshita T, Rosse W, Socié G; International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood 2005;106:3699-709. doi: 10.1182/blood-2005-04-1717
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  4. Mortazavi Y, Tooze JA, Gordon-Smith EC, Rutherford TR. N-RAS gene mutation in patients with aplastic anemia and aplastic anemia/ paroxysmal nocturnal hemoglobinuria during evolution to clonal disease. Blood 2000;95:646-50.