A presentation By Abedelaziz Taha Hammash supervisor \ Mr A presentation By Abedelaziz Taha Hammash supervisor \ Mr. Wael Al-laithy Case study
A 35 year-old women presented with a two month history of increasing fatigue and abdominal fullness with accompanying loss of appetite. There was no history of bleeding manifestation, on examination the patient had pallor and splenic enlargement. Physical examination was otherwise unremarkable, CBC findings included Hb of 7.5g/dl, RBCs 3.5 million cells per microliter, and total leukocyte count of 27900 cell per microliter, and a platelet count of 364000 platelets per microliter.
The peripheral blood smear showed the folloing features: 26% blasts, 7% myelocytes, 6% metamyelocytes, 4%basophils, 2% eosinophils, 10%lymphocytes, 45% neutrophils(band & segmented). The blasts showed scant cytoplasm, round nuclei with fine chromatin, and 2-4 nucleoli. Hypo- and hypersegmented neutrophils. Many giant platelets and platelet aggregates were seen.
Blasts with cytoplasmic blebs. Abnormal, large platelets. B.M smear showed abnormal megakaryocytes and marrow fibrosis. Immunophenotyping showed strong CD 61 positivity in megakaryocytes. The Philadelphia chromosome was detected in the patient’s karyotype.
Blasts with cytoplasmic blebs
Abnormal large platelets
The case is Megakaryocytic blast crisis (AML M7)
Discussion The diagnosis of blast transformation of CML is made by a blast count of >20% in peripheral blood and/or marrow, the natural course of transformation from chronic phase to blast phase takes four years. The diagnosis of AML M7 can be considered when peripheral blood shows blasts with cytoplasmic blebs and platelets abnormalities, and BM show abnormal megakaryocytes associated with marrow fibrosis. Confirmation of megakaryocytic lineage is by demonstration of platelet lineage- specific markers, namely CD41, CD42b, CD61, CD62. It’s uncommon to find normal platelet count in CML blastic crisis (as in our case).
background Phases of CML: Chronic phase Accelerated phase Blast phase
In blastic phase the blast should exceed 30% in peripheral blood (blastic crisis), which is an evolution of CML that give rise to acute phase (acute megakaryoblastic leukemia in our case), this is manifested by progression of anemia, neutropenia and thrombocytopenia. But our case here is distinguished by normal platelet count, it could be due to megakaryocytic proliferation seen in this condition.
(AML (M7 Acute megakaryoblastic leukemia (AMKL) is life- threatening leukemia in which malignant megakaryoblasts proliferate abnormally and injure various tissues. Megakaryoblasts are the most immature precursor cells in a platelet-forming lineage; they mature to promegakaryocytes and, ultimately, megakaryocytes which give platelets.
Treatment Chemotherapy Tyrosine kinase inhibitor Alpha-Interpheron Stem cell transplant
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