Cutaneous extramedullary hemopoiesis in chronic myeloproliferative and myelodysplastic disorders

Transforming growth factor–β overexpression in cutaneous extramedullary hematopoiesis of a patient with myelodysplastic syndrome associated with myelofibrosis

“…9-12 Cutaneous EMH has been occasionally described, primarily in association with chronic idiopathic myelofibrosis, [13][14][15] but it has not been reported in the setting of MDS with myelofibrosis. Compared with MDS without myelofibrosis, MDS with myelofibrosis is characterized by the following hematologic features 3-5,16-18 : (1) marked fibrosis of the bone marrow; (2) increased numbers of small hypolobulated megakaryocytes in the bone marrow; (3) modest or absent visceral enlargement; and (4) poor prognosis as a result of high incidence of bone-marrow failure. Our patient fulfilled these findings and was given the diagnosis of MDS with myelofibrosis.…”

“…The prognosis of patients with EMH is poor. 2 Koch et al 19 reported that the median survival of patients with nonhepatosplenic EMH in chronic idiopathic myelofibrosis is 13 months. At the time that we observed enlargement of the multiple cutaneous nodules over the trunk, our patient was unresponsive to a variety of treatment regimens and died from bone-marrow failure within 5 months.…”

“…About 30 cutaneous EMH cases associated with myelofibrosis have been reported. 1,2 The diagnosis of cutaneous EMH has been made histopathologically, with immunohistochemistry confirming the presence of differentiating hematopoietic cells intermixed with dermal components. Myelodysplastic syndrome (MDS) is a clonal hematopoietic stem cell disorder characterized by multilineage dysplasia and pancytopenia as a result of ineffective hematopoiesis.…”

Cutaneous myeloid sarcoma of the penile foreskin

“…In adults, it is described mostly in myelofibrosis; although it may occur rarely in CML. New evidence suggests that cutaneous EMH in chronic myeloid disease should be considered as metastasis of the abnormal neoplastic cells and subsequent differentiation along divergent myeloid lineages (9). Recent demonstration of the characteristic Janus kinase 2 gene mutation in the cutaneous lesion of a patient with primary myelofibrosis also supports the metastasis hypothesis (10).…”

“…The erythroid precursors were scanty and megakaryocytes numerous including abnormal forms. Using the LSI bcr/ abl dual color dual fusion probe (Vysis) for interphase FISH, 83.5% (167/200) of the nuclei showed a fusion signal indicative of translocation (9,22). Oral imatinib (400 mg/day) therapy was started and 12 months hence the patient is in hematological remission with no evidence of extramedullary disease.…”