Subcutaneous Extramedullar Hematopoiesis in a Patient with Secondary Myelofibrosis Following Polycythemia Vera

Abstract

We describe the case of a 73-year-old woman with secondary myelofibrosis who developed subcutaneous extramedullary hematopoiesis. Although extramedullary hematopoiesis has been generally observed in primary myelofibrosis, in this case it was seen in myelofibrosis secondary to polycythemia vera. Histological examination of the subcutaneous nodule revealed that the lesion included cells from the myeloid and megakaryocytic series. The skin lesion almost disappeared after treatment with hydroxyurea. We report here this rare manifestation in secondary myelofibrosis including a review of literature.

A case of extramedullary hematopoiesis presenting as hemorrhagic panniculitis and evolving in acute myeloid leukemia

“…Cutaneous EMH is a rare complication, resulting from migration of abnormal neoplastic hemopoietic precursor cells into the skin, with subsequent differentiation into divergent cell lineages . It is often seen in the context of myelofibrosis evolving from chronic myeloid diseases, in polycythemia vera or essential thrombocytosis . Apart from the association with hematologic disorders, EMH has also been reported within cases of pyogenic granuloma .…”

A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

“…Very few of them developed in the setting of secondary MF. Tanaka et al [12] reported four cases of secondary MF (three PV and one essential thrombocythemia) presenting with cutaneous manifestations, in their literature review. Whether due to primary or secondary MF, the skin lesions range from reddish-purplish macules, papules or erythematous plaques to nodules, ulcers and bullae.…”

Fine needle aspiration of an axillary lymph node in a patient suspected of having metastatic cancer of unknown primary

“…5,7 EMH occurs most frequently in the spleen, liver and lymph nodes, however it has also been described in the thyroid, endometrium, central nervous system, subcutaneous tissue, pleura, retroperitoneum, mediastinum, breast and kidney. [1][2][3][4][5][6][7]9,10,[13][14][15][16][17][18][19][20][21] Patients often present with symptoms related to the location of the EMH. EMH is seen mostly in neonates and the elderly.…”