PMC4857264_01
Morphology and CBC results of patient 1 with autoimmune myelofibrosis. PB and BM morphology. (a) PB smear revealed normocytic anemia with very rare teardrop cells, and mild leukopenia with no evidence of leukoerythroblastosis (Wright stain: x50). (b) BM aspiration smear showed normal M:E ratio and no significant dysplasia in trilineage hematopoietic cells except occasional erythroid cells showing irregular nuclear contours, and unremarkable granulocytic and megakaryocytic lineages (Wright staining: x50). (c and d) BM core biopsy displayed hypercellularity (95%) with panhyperplasia, megakaryocytic proliferation with focal clustering, and interstitial benign lymphoid aggregates (H&E stain: x20). (e) Phospho-STAT5 immunostain on the core biopsy showed negative staining of megakaryocytic nuclei. (f) Reticulin stain on core biopsy showed moderate increase in reticulin fibers without increase in collagen fibers (myelofibrosis grade 2 on a scale of 0-3). (g) CBC values were reported for patient 1 for a range of 32 months. Types of treatment were indicated by different color bars that showed when treatment was given (light blue: prednisone; yellow: methylprednisolone; magenta: rituximab; green: mycophenolate; and red: IVIg).. CBC: complete blood count; PB: peripheral blood; BM: bone marrow; H&E: hematoxylin and eosin; M:E ratio: mass-to-charge ratio; IVIg: intravenous immunoglobulin.
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Hematologic parameters are shown in Figure 1(g). A BM biopsy showed a hypercellular BM (95%) with panhyperplasia associated with moderate reticulin fibrosis (grade 2+) (Figure 1(f)).
PMC4857264_02
Morphology and CBC results of patient II with autoimmune myelofibrosis. BM morphology: (a) BM aspiration smear revealed slightly low M:E ratio, mild megaloblastoid changes in erythroid cells, and unremarkable granulocytic and megakaryocytic lineages (Wright stain: x50). (b) BM core biopsy displayed hypercellularity (95%) with panhyperplasia, megakaryocytic proliferation with focal clustering (H&E stain: x20). (c) Phospho-STAT5 immunostain on the core biopsy showed negative staining of megakaryocytic nuclei. (d) Reticulin stain showed diffuse and dense increase in reticulin fibers without increase in collagen fibers (myelofibrosis grade 2 on a scale of 0-3). (e) Routine CBC tests were performed and results are displayed for a period of 42 months. Types of treatments were indicated by different color bars that showed when treatment was given (orange: dexamethasone and light blue: prednisone).. CBC: complete blood count; BM: bone marrow; H&E: hematoxylin and eosin; M:E ratio: myeloid to erythroid ratio
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PMC4857264_02_10.1177_2050313X13498709-fig2.jpg
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Reticulin staining showed moderate reticulin fibrosis (Figure 2(a)-(d)). The patient required RBC transfusions every 1-2 weeks for a full year and then, suddenly became transfusion independent with complete normalization of hemoglobin levels (Figure 2(e)).