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

114 - 116, 15.05.2016

Rafet Eren , Tayfur Toptas İsik Atagunduz Tülin Fıratlı Tuglular

https://doi.org/10.5472/MMJcr.2902.02

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) monocyte

clone 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

Paroksismal noktürnal hemoglobinüriden evrilen akut promyelositik lösemi olgusu: Nadir bir birliktelik

Abstract

Bilinen bir hastalığı olmayan ve dispne, demir eksikliği anemisi, pansitopeni, splenomegali ve anormal koagülasyon testleri ile başvuran 31 yaşında kadın hasta hastaneye yatırıldı. Hematoloji değerlendirmede hücrelerin %71’inin akut promyelositik lösemi (APL) ile uymlu olduğu saptandı ve hastanın t(15;17) translokasyonu pozitif gelerek APL doğrulandı. All-trans retinoik asit (ATRA) tedavisi hemen başlandı ancak hastada ağır dispne bulguları gelişti. Bu durum dolaşımsal ve kardiyak arreste yol açtı ve ölümle sonuçlandı. Hastanın ölümünden sonra sonuçlanan
FLAER tetkikinde paroksismal nokturnal hemoglobinüri tanısını
doğrulayan 82% monosit klonu saptandı. Nokturnal hemoglobinüri
hastalarında nokturnal hemoglobinüri dışı klonlardan lösemi
gelişebilmektedir. Aşikar yaygın damar içi pıhtılaşması bulguları
olmayan ağır trombositopenik bir hastada agresif antikoagülasyonla
kontrol edilemeyen katastrofik tromboembolik olaylar eşlik eden
nokturnal hemoglobinüriye işaret edebilir.

Keywords

Lösemi, Promyelositik, Akut, Hemoglobinuri, Proksismal

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