| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 142 |
Submitter(s): Friederike H. Kreisel.
Clinical history
This is the case of a 86-year old female who presented to the hospital with bilateral axillary lymphadenopathy and abdominal pain. Her past medical history was significant for hypertension, hypothyroidism, glaucoma, and osteoporosis. Her medications included: Xalatan eye drops, Timoptic eye drops, Trusopt eye drops, Fosamax, Lasix 40 mg daily, KayCiel 10 mEq daily, Atenolol 100 mg p.o. daily, Plendil 10 mg daily, Synthroid 112 mcg daily. Family history was significant for an identical twin sister who died one year earlier of "some sort of leukemia". A CBC revealed: WBCs 20.4 x 103/mcl with 47% monocytes, hemoglobin 9.3 g/dl, hematocrit 26.9%, platelets 79. A CT of the chest and abdomen revealed extensive bilateral axillary and retrocrural lymphadenopathy. The spleen wass reported to be normal in size. The patient died one year later due to hemorrhage due to acute onset of melena. A routine CBC two months prior to her death showed persistent bicytopenia and a WBC of 7.8 x 103/mcl with 47% monocytes.
Details of gross/microscopic pathology:
Both a bone marrow biopsy and excisional biopsy of an enlarged axillary lymph node were performed.
The bone marrow core (0.8 x 0.2 cm) was received in 10% neutral buffered formalin. The specimen was decalcified in EDTA. Review of both the core biopsy and corresponding Wright-Giemsa stained aspirate showed a markedly hypercellular marrow with myeloid and megakaryocytic hyperplasia and dysplasia. Approximately 13% of nucleated cells represented monocytes. Dysgranulopoietic features included abnormal lobation and granulation, and monocytoid change. Dysmegakarypoietic changes included micromegakaryocytes and hypolobation. Myeloblasts were not increased.
The axillary lymph node measured 1.2 cm in diameter. One portion was fixed in 10% neutral buffered formalin and some fresh material was send for flow cytometry. Microscopic examination showed extramedullary hematopoiesis (EMH) with aggregates of erythroid precursors, maturing myeloid and monocytic cells, and megakaryocytes. No increase in blasts was seen.
Immunophenotype (flow cytometry/immunohistochemistry):
Lymph node: Flow cytometry was negative for a clonal B-cell population. Special stains showed maturing myeloid elements to be Leder+, CD43+, and myeloperoxidase+. A smaller subset expressed CD117. CD34 was negative in myeloid cells. CD68 and lysozyme highlighted mature histiocytes within sinuses and monocytic cells in aggregates of EMH. Immunohistochemistry for CD4 and CD56 was negative for diffuse areas of plasmacytoid dendritic cells.
Cytogenetics:
BM: Normal female karyotype.
Molecular analysis:
Not performed.
Interesting feature(s) of submitted case:
Rare case of chronic myelomonocytic leukemia with concurrent nodal presentation.
Proposed diagnosis:
Chronic myelomonocytic leukemia with associated extensive lymphadenopathy due to extramedullary hematopoiesis, likely representing involvement by chronic myelomonocytic leukemia.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Stains performed by the panel: CD117-, CD68PGM1 weakly +, CD68-KP1+, NPM- (+in nuclei), CD163+, MPO focally+
Images:
 |
Figure 1 |
 |
Figure 2 |
 |
Figure 3 |
 |
Figure 4 |
 |
Figure 5 |
 |
Figure 6 |
 |
Figure 7 |
 |
Figure 8 |
 |
Figure 9 |
 |
Figure 10 |
Back to Top
Back to Cases by Session
Back to Cases by Contact Submitter