PMC3420541_01
Primary myelofibrosis, prefibrotic stage: (a) peripheral blood, thrombocytosis with giant platelets, Wright-Giemsa stain, Ob. 100x, immersion oil. (b) bone marrow biopsy with mildly hypercellular bone marrow, hematoxylin and eosin stain, Ob. 40x. (c) bone marrow aspirate, dysmegakaryopoiesis, Wright Giemsa stain, Ob. 100x, immersion oil. (d) Reticulin stain, mildly increased reticulin deposition. Ob. 50x.
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fig1
The peripheral blood smear showed neutrophilia with a subset of hypersegmented neutrophils, (RBCs) with moderate anisopoikilocytosis, and also thrombocytosis with a subset of giant platelets (Figure 1(a)). The bone marrow was mildly hypercellular (50%) (Figure 1(b)), had trilineage hematopoiesis, a normal M : E ratio, megakaryocytic hyperplasia (9.5 megakaryocytes/hpf of 40x), and dysmegakaryopoiesis (Figure 1(c)). A reticulin stain was remarkable for a mildly increased (2+/4) deposition of reticulin fibers (Figure 1(d)).
Primary myelofibrosis, fibrotic stage: (a) peripheral blood smear, Wright-Giemsa stain, blast (arrow), Ob. 100x immersion oil. (b) Hypercellular bone marrow biopsy, Hematoxylin and eosin stain, Ob. 50x (c) bone marrow biopsy with markedly increased reticulin deposition. Ob. 50x, (d) Anti-CD20 antibody highlights a large subset of positive lymphoid cells in a lymphoid aggregate, Ob. 50x. (e) Anti-CD3 antibody highlights a small subset of CD3+ T-cells in a lymphoid aggregate, Ob. 50x. (f) Anti-Cyclin D1 antibody highlights a small subset of positive nuclei in a lymphoid aggregate, Ob. 50x.
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August 2010 when the WBC reached 59.3 x 109/L, and a leukoerythroblastic picture was noted (Figure 2(a)) with a white blood differential including segmented neutrophils 80%, bands 6%, lymphocytes 2%, monocytes 3%, eosinophils 1%, basophils 1%, meta/myelocytes 2%, and blasts 5%. A subset of neutrophils had hypersegmented nuclei; a few hypogranular neutrophils were also noted and there was associated macrocytic anemia (hemoglobin 12.6 g/dL, MCV: 113.1 fL) and tear drop RBCs. The bone marrow aspirate sampled was a "dry tap," but the biopsy was markedly hypercellular (cellularity 95%) with megakaryocytic hyperplasia (>10 megakaryocytes/hpf) and showed dysmegakaryopoiesis, a few small ill-defined intertrabecular lymphoid aggregates (Figure 2(b)) and also marked fibrosis (4+/4) (Figure 2(c)). T cells (Figure 2(e)). The B cells were positive for bcl2 and negative for CD5 and CD10. Few scattered cyclin D1+ nuclei were noted in some of these lymphoid aggregates, raising the suspicion of mantle cell lymphoma (Figure 2(f)).
Acute myeloid leukemia and associated bone marrow involvement by mantle cell lymphoma: (a) peripheral blood smear, Wright-Giemsa stain, blasts, Ob. 100x, immersion oil. (b) Bone marrow biopsy, Ob. 20x, anti-CD34 antibody highlights positive blasts; the lymphoid cells are negative. (c) Bone marrow biopsy, hematoxylin and eosin stain, Ob. 50x, lymphoid aggregate and blasts. (d) Bone marrow biopsy, anti-CD20 antibody highlights a large subset of positive cells in a lymphoid aggregate, Ob. 20x. (e) Bone marrow biopsy, anti-CD3 antibody highlights a small subset of positive cells in a lymphoid aggregate, Ob. 20x. (f) Bone marrow biopsy, anti-Cyclin D1 antibody highlights a large subset of positive cells in a lymphoid aggregate, Ob. 20x.
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In 2011, while presenting with symptoms suggestive of a respiratory infection, the CBC showed leukocytosis (WBC 40.3 x 109/L), hemoglobin of 11 g/dL, MCV 115 fL, platelets 215 x 1012/L (Table 1), and the peripheral blood smear was remarkable for 69% blasts (Figure 3(a)). The hypercellular bone marrow biopsy (95%) had approximately 70% CD34+ blasts (Figure 3(b)) and several small-to-medium size lymphoid aggregates (Figure 3(c)), consisting of small to medium sized lymphoid cells. T-cells (Figure 3(e)). The B-lymphoid cells had variable expression of cyclin D1 (Figure 3(f)) and a low proliferation rate (approx. 5% to focally 10-20%).
PMC3420541_02