Myelodysplastic Syndromes

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Acquired Pelger-Huet Anomaly associated with drugs • a predominance of uni-lobed neutrophils (with occasional bi-lobed forms present); in some cases many band forms may also be seen • Abnormally coarse nuclear chromatin • uniformity in appearance of the neutrophil population • ABSENCE OF OTHER MORPHOLOGIC DYSPLASIA Dyserythropoiesis Dysgranulopoiesis Dysmegakaryopoiesis Chemotherapy/bone marrow Chemotherapy/bone marrow Chemotherapy/bone marrow regeneration regeneration regeneration Vitamin B12/folate Vitamin B12/folate Vitamin B12/folate deficiency deficiency, and rarely deficiency, and rarely (some cases) pyridoxine (B6) deficiency pyridoxine (B6) deficiency Infections (parvovirus, HIV) Infections (HIV) Infections (HIV) Autoimmune conditions Autoimmune myelofibrosis Post-transplant Post-transplant Medications, alcohol and other toxins, heavy metals (e.g. arsenic) etc. Rapid erythroid proliferation in response to anemia; erythropoietin therapy Aplastic anemia/PNH Paraneoplastic Paraneoplastic myelofibrosis Medications (e.g. tacrolimus, mycophenolate mofitel, gancyclovir, purine analogs) Exogenous (G-CSF) or endogenous (e.g. sepsis) cytokines Hemophagocytic Hemophagocytic lymphohistiocytosis lymphohistiocytosis Congenital dyserythropoietic Congenital (e.g. Fanconi anemias anemia) Hemophagocytic lymphohistiocytosis Transient abnormal myelopoiesis of Down syndrome; other congenital disorders Copper Deficiency • Granulocytes: −left shifted −vacuolization of precursors • Erythroid series: −Left shifted with mild megaloblastic changes and terminal dyserythropoiesis −vacuolated cytoplasm −ringed sideroblasts • Megakaryocytes: usually normal • Variable marrow cellularity • Blasts are generally NOT increased. G-CSF may result in increased blasts and neutrophil dysplasia 14
The patient is a 56 year old man who is 2 years post orthotopic liver transplant. The patient is receiving tacrolimus and mycophenolate mofetil for immunosuppression. CBC shows: WBC 2.6 x10 9 /L; neuts 1.5 x10 9 /L Hgb 8.9 g/dL MCV 79 fL Platelets 170x10 9 /L Pelger-Huet Neutrophils • Inherited Pelger-Huet anomaly • Myeloid neoplasm, such as MDS or AML • Recent chemotherapy • Other medications, including mycophenolate mofetil, tacrolimus, gancyclovir, and even trimethoprimsulfamethoxazole • Infection associated (uncommon, but occurs) In this case, neutropenia resolved 4 weeks after decreasing mycophenolate mofetil dose 15
Page 1 and 2: Myelodysplastic Syndromes Joan E. E
Page 3 and 4: WHO MDS Classification • Dysplasi
Page 5 and 6: Neutrophil dysplasia • Hypo-, agr
Page 7 and 8: MDS cytogenetics Presumptive diagno
Page 9 and 10: Hypocellular MDS • Proper classif
Page 11 and 12: Paryoxysmal Nocturnal Hemoglobinuri
Page 13: The patient is a 32 year old taxi d

Myelodysplastic Syndromes

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