| Session: Ph- chronic myeloproliferative disease |
| Case number: 203 |
Submitter(s): Marcus Kremer, Falko Fend.
Clinical history
31 year old female with a history of an acute myeloid leukemia (M5b FAB), diagnosed one year prior to the presented bone marrow biopsy. The patient achieved complete remission after chemotherapy (induction I + II with cytarabine, idarubicin, etoposid). Relapse one year later with cutaneous chloromas, and bone marrow infiltration. Striking megakaryocytic proliferation was noticed at that time. After chemotherapy of the relapse, the patient achieved CR but shows the presented BM morphology of highly increased dysplastic megakaryocytes, fibrosis with profound hypoplasia of the remaining lineages.
Details of gross/microscopic pathology:
BM biopsy, formalin fixed and EDTA-decalcified.
Biopsy shows a highly cellular bone marrow, with an increased amount of dysplastic megakaryocytes, fibrosis, and relative hypoplasia of the erythroid and granulocytic lineage. Blasts of the treated AML (M5b FAB) are currently not detectable.
Immunophenotype (flow cytometry/immunohistochemistry):
The initial blast phenotype was MPO, CD33, CD13, and partially CD117, CD56, CD7, CD64, CD4, CD15.
Cytogenetics:
46, XX (21).
Molecular analysis:
JAK2 V617 mutation negative.
Interesting feature(s) of submitted case:
The presented case possibly represents a transformation of an underlying myeloproliferative syndrome (MPS unclassifiable, JAK2 negative), which became apparent in the first relapse of the AML. The underlying MPS is characterized by an excessive proliferation of highly atypical megakaryocytes, accompanied by a marked fibrosis.
Proposed diagnosis:
Secondary AML (M5b FAB) as a transformation of an unclassified myeloproliferative syndrome (JAK2 negative).
Panel diagnosis:
agree with proposed diagnosis
Comments:
Images:
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