| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 212 |
Submitter(s): Guang Fan, Rita Braziel, Ken Gatter, Marc Loriaux, James Huang.
Clinical history
46 year old woman presented with right axillary lymphoadenopath and right inguinal lymphoadenopathy with history of Hepatitis C. The right axillary node biopsy was done at 5/5/2005 to rule out infectious process.
Patient had one bone marrow biopsy was done in 1996 due to leukocytosis and pathology report stated normal cellular bone marrow with trilineage hematopoiesis.
We suggested peripheral blood and bone marrow work up, after lymph node biopsy was reviewed. The CBC on 6/8/05 showed WBC 10.5, Hb 9, Hct 28.3, MCV100, PMN 67%, lymphs 25, and monos 8%. Bone marrow biopsy showed hypercellular bone marrow with marked erythroid hyperplasia and dysplasia. The
diagnosis was myelodysplastic syndrome, refractory anemia without increase in blasts.
5/4/06 peripheral blood showed rare blasts. 5/8/06 bone marrow biopsy revealed hypercellular bone marrow with trilineage dysplasia, RAEB-II (12% blasts). 11/29/06 and 12/29/06 bone marrow biopsies showed same feature without any improvement. Even though patient received 4 cycles of chemotherapy with decitabine 20 mg/sq from July 2006 to November 2006.
1/10/07 patient had severe pancytopenia. Bone marrow biopsy demonstrated hypercellular bone marrow with 60% blasts, which represent acute myeloid leukemia transformation.
Details of gross/microscopic pathology:
All biopsies were fixed in formalin.
Right axillary lymph node biopsy on 5/5/05 showed medullary mononuclear form infitrates, which extend to surround fatty tissue. The cells had eosinophilic and granular cytoplasm. Scattered residual germinal centers were seen.
6/8/05 peripheral blood showed marked anisocytosis and monocytosis. Blasts are not seen. Neutrophils are not dysplastic. Platelets are adequent. Aspirate and biopsy were hypercellular with prominent erythroid dysplasia.
5/8/2006, 11/29/2006, and 12/29/2006 peripheral blood showed rare circulating blasts, anisocytosis, and dysplastic neutrophils. Bone marrow aspirate and biopsy showed hypercellular bone marrow with trilineage dysplasia and increased blasts. No increased ringed sideroblasts were seen.
1/10/06 Peripheral blood showed pancytopenia. Marrow showed 60% blasts.
Immunophenotype (flow cytometry/immunohistochemistry):
Lymph node biopsy: The myeloid precursors are positive for MPO, CD68, CD43, and partial CD45; negative for CD117 and CD34.
Bone marrow blasts by flow: CD45 dim, CD34, CD117, HLA-DR, CD13 dim, CD33 dim.
Cytogenetics:
normal.
Molecular analysis:
JAK2 and Kit mutations were negative.
Interesting feature(s) of submitted case:
1. Long time mild leukocytosis and recurrent infections.
2. Lymphoadenopathy as first evidence of myeloid neoplasm.
3. Patient may had long time myeloproliferative disorder, then progress to myelodisplastic disorder, then acute myeloid leukemia.
4. Normal cytogenetic and negative molecular test result imposed additional diagnostic chanllenge.
Proposed diagnosis:
Myeloproliferative/myelodysplastic syndrome with lymph node involvement and transformation to acute myeloid leukemia.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Stains performed by the panel: CD68 PGM1 +, MPO+, NPM- (+in nuclei)
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