Eritroleucemia – cauză rară de leucemie acută

A 44-year-old man presented for repetitive epista­xis and marked physical asthenia. The clinical exam revealed only profuse sweating, moderately pale teguments and hepatosplenomegaly.

The CBC at admission showed severe pancytopenia: WBC 3800/mm³, hemoglobin 6.9 g/dl and platelet count 17,000/mm³. The biochemistry was unremarkable, with the exception of increased LDH (> 5x normal) and mild hepatocytolysis.

The peripheral blood smear revealed leukoerythroblastosis, with 13% blasts and 35 erythroblasts per 100 nucleated elements. The blast cells were medium-large in size, with basophilic cytoplasm, thin chromatin, nucleoli, some with granulation and rare with Auer bodies.

The bone marrow smear described hypercellular blood marrow, with 10% blast cells and markedly increased erythroblastic lineage (79%). The erythroblastic cells were large, with megaloblastic features, abundant basophilic cytoplasm with cytoplasmic projections and vacuolization, immature nuclear chromatin, and occasional nucleoli. The PAS staining was suggestive for AML6⁽¹⁾.

Immunophenotypic profile: glycophorin A positive, CD71 positive and CD33 positive, being suggestive for erythroleukemia – FAB classification⁽²⁾ – AML6. This subtype is an exceptionally rare category of acute leukemia (3-8%), usually accompanied by unfavorable prognosis and evolution⁽¹⁾.

The cytogenetic exam revealed loss of the long arm of chromosome 5 – del (5q), associated with an adverse outcome⁽²⁾.

The patient was successfully treated according to the “3+7” induction protocol⁽³⁾ and then referred for consolidation with allogeneic bone marrow transplantation, performed from a matched unrelated donor, fortunately with a surprising favorable evolution.  
 

Conflict of interests: The authors declare no conflict of interests.