| Session: Myelodysplastic syndrome |
| Case number: 007 |
Submitter(s): Gerald M. Penn.
Clinical history
The patient is an 89 year old male with previous history of type-2 diabetes, stroke with expressive aphasia, hypothyroidism, renal insufficiency, and atrial fibrillation. He is admitted to the hospital because of hyperglycemia, pneumonia, and decreasing Hgb and PLTs over the previous four months. On admission, his Hgb was 6.3 g/dL, PLTs 65 K/uL, and WBC 13 K/uL with 92% PMNs. After treatment with IV antibiotics and transfusion of packed red cells, his CBC showed: WBC 5.2 K/uL, Hgb 9.2 g/dL, MCV 88.6 fL, and PLTs 36 K/uL. The differential of nucleated cells was 72% PMNs, 19% Lymphs, 7% Monos, and 2% Eos. His serum total protein was 9.3 g/dL and a serum protein electrophoresis with immunofixation showed 3.6 g/dL monoclonal IgGK. The serum calcium was 8.5 mg/dL.
Details of gross/microscopic pathology:
An evaluation of the peripheral blood smear demonstrates hypogranular pelgeroid PMNs, leukoerythroblastosis, and rare plasma cells.[figure1]A bone marrow aspirate smear shows plasmacytosis and 4.5% blasts.[figure2][figure3]The aspirate clot sections demonstrate plasma cell nodules with Kappa light chain restriction.[figure4][figure5]
Immunophenotype (flow cytometry/immunohistochemistry):
Flow cytometry shows the increased myeloblasts with down-regulation of CD45. In addition, the myeloid cells have decreased side-scatter and down-regulation of CD16 and CD64. In contrast, the plasma cells have acquired CD56.[figure6][figure7]Further evaluation of the aspirate clot sections shows that the plasma cell nodules are weakly positive for Cyclin D1 and the CD34 positive blast cells are scattered throughout the non-myeloma marrow.[figure8]
Cytogenetics:
Cytogenetics showed: 47,XY,+8[12]/47,XY,+11[5]/46,XY[5].
Molecular analysis:
FISH analysis demonstrated trisomy 8 in 85% of nuclei and trisomy 11 in 17% of nuclei.[figure9]
Interesting feature(s) of submitted case:
This case demonstrates the importance of using multiple technologies for evaluating bone marrow samples, so that the appropriate therapy may be selected.
Proposed diagnosis:
Myeloma with Refractory Cytopenia and Multilineage Dysplasia.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Additional information from the submitter: Patient was treated with rbc transfusions only and died at home in 3/06. The bone marrow aspirate differential was: Myeloid 69%, Erythroid 11.5%, Lymphoid 6.5%, Monocytes/histiocytes 3.5%, Plasma cells 5%, and Blasts 4.5%. No splenomegaly/organomegaly was detected.
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