| Session: Chronic myelogenous leukemia |
| Case number: 120 |
Submitter(s): Sergey D. Popov, Margarita Palutke.
Clinical history
A 16 year old boy presented at an outside hospital with fatigue and fever. A CBC showed a white cell count of 159,000/cumm which were mostly blasts. Immunophenotyping results showed this to be precursor B acute lymphoblastic leukemia. He was started on prednisone but the count kept rising. He was transfered to Children's Hospital #1 of St.Petersburg with a WBC of 206,000 with 85% blasts, 2% metamyelocyte, 2% eosinophils, 1% monocytes, 4% lymphocytes, anemia and thrombocytopenia. Immunophenotyping was repeated on the blood showing it to be acute myelogenous leukemia.A bone marrow examination showed two populations of blasts; myeloid and lymphoid. He was treated with prednisone, Rubomycin, Gleevec, Cytozar and Vipezid all without response and then was put on an AML-BFM protocol. He became cytopenic and died two months after first admission.
Details of gross/microscopic pathology:
The bone marrow aspirate showed two morphologically different blasts. The majority were larger blasts with abundant cytoplasm, many with cytoplasmic granules. A minor population cosisted of smaller blasts with a high nuclear cytoplasmic ratio and no granules. In addition there were some promyelocytes and many eosinophils with bsophilic granules and occasional basophilic myelocytes. Erythroblasts showed some dysplasia. There was no bone marrow biopsy.
Immunophenotype (flow cytometry/immunohistochemistry):
The initial flow cytometry at another hospital showed the blasts to be CD19,CD10, CD79a, Dr and TdT positive with weak coexpression of CD13 and CD33.
The repeat flow cytometry on the bone marrow at Children's Hospital #1 showed two populations. The predominant one was strongly CD13 and CD33 positive and weakly CD117 positive, partially myeloperosidase and DR positive with 50% of cells TdT positive. There was weak coexpression ofCD19. The smaller population was CD19, CD79a, and CD10, weakly CD22, Dr and 100% TdT positive, with weak CD13, CD33, and CD34 coexpression.
Cytogenetics:
Cytogenetic analysis at initial presentation showed that 100% of cells had t (9;22).
Molecular analysis:
There was a BCR-ABL (B2A2) fusion gene on the type seen in chronic myelogenous leukemia.
Interesting feature(s) of submitted case:
1. Bilineal acute leukemias are rare. One may follow the other after chemotherapy. In this case because of the short interval between prednisone therapy for ALL and the appearance of AML this would not be the case. The two leukemic cell lines must have coexisted from the beginning.
2. Both leukemias arose from a Ph positive chronic myelogenous leukemia, a stem cell disorder, which was not detected initially, and rapidly progressed to the acute phase, in this case a bilineal type.
Proposed diagnosis:
Bilineal acute leukemia, myeloid and B lymphoid, arising from a Ph + chronic myelogenous leukemia, previously not detected.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Panel comment: Both clones may derive from a single neoplastic stem cell.
Images:
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