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Hemophagocytic Syndrome

View larger version (116K): [in this window] [in a new window]   Figure 1. Patient is a 7 week old who presented with a two week history of greater than 10 episodes per day of diarrhea. A CBC on admission revealed an H/H of 9.3/27, WBC of 1200/uL and platelet count of 32,000/uL. Serum ferritin was markedly elevated at 6250. A diagnosis of hematophagocytic lymphohistiocytosis (HLH) was made. An 18 week course of cyclosporine, dexamethasone and VP-16 was initiated with eventual full recovery. A number of abnormalities are seen on the peripheral smear including marked thrombocytopenia, mild anisocytosis and poikilocytosis with spherocytes, occasional fragmented RBCs and polychromatrophilic RBCs.

View larger version (105K): [in this window] [in a new window]   Figure 2. A neutrophil with toxic granulation is noted in this view. Few platelets are present. Burr and spur cells are also noted in addition to the spherocytes and polychromatophilic RBCs.

View larger version (160K): [in this window] [in a new window]   Figure 3. A low power view of the aspirate show a hypercellular marrow with a marked increase in megakaryocytes. Islands of erythroid precursors are also prominent.

View larger version (117K): [in this window] [in a new window]   Figure 4. Three macrophages ingesting platelets, mature RBCs and erythroid precursors and myeloid precursors in addition to other cellular debris are seen in this view.

View larger version (80K): [in this window] [in a new window]   Figure 5. There is prominent phagocytosis of erythroid precursors in this view.

View larger version (89K): [in this window] [in a new window]   Figure 6. Multiple erythroid precursors in addition to the single mature RBC are seen in the cytoplasm of the macrophage.

View larger version (166K): [in this window] [in a new window]   Figure 7. The marrow biopsy is approximately 95% cellular. Megakaryocytes are prominent.

View larger version (171K): [in this window] [in a new window]   Figure 8. At a higher magnification of the previous image it is apparent that there is a hyperplasia of both the erythroid and megakaryocytic lineages.

View larger version (135K): [in this window] [in a new window]   Figure 9. The erythroid hyperplasia is evident in this view. Hemophagocytosis can be seen in several macrophage(intracytoplasmic nucleated RBCs are present).

View larger version (137K): [in this window] [in a new window]   Figure 10. There is a marked increase in Prussian Blue staining of the single spicule noted in this image.

View larger version (144K): [in this window] [in a new window]   Figure 11. The iron stain (Prussian Blue) outlines the single macrophage seen in this view. At least three hematopoietic cells can also be seen in the macrophage cytoplasm.

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This Article Figures Only Services Similar articles in this journal Alert me to new content in the Image Bank Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Lazarchick, J. Search for Related Content PubMed Articles by Lazarchick, J. Related Collections Hemolytic Disorders Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Articles Social Bookmarking                   What's this?