Idiopathic Myelofibrosis with Nodal, Serosal and Parenchymatous Infiltration

Abstract

Idiopathic myelofibrosis (IMF) is a breakpoint cluster region rearrangement-negative chronic myeloproliferative disease with progressive bone marrow fibrosis. We report a female patient (65 years old) who was admitted to our hospital in 1996. Trephine bone marrow biopsy revealed diffuse fibrosis with atypical multilobulated megakaryocytes. A cellular phase of IMF was diagnosed. Three years later, despite being at intermediate risk, the patient developed generalized lymphadenopathy and multiple sclerosing tumors throughout the peritoneum and retroperitoneum. Biopsy specimens from these tumors revealed sclerosing hematopoietic infiltrates. The present case demonstrates that IMF not only can progress to acute leukemia but can also spread uncontrollably, or ‘metastasize’ with extensive sclerosing hematopoietic tumors throughout the body.

Idiopathic myelofibrosis with disseminated hepatosplenic, mesenteric, renal and pulmonary extramedullary haematopoeisis, portal hypertension and tuberculosis: initial presentation and 2 years follow-up

“…Unusual sites or disseminated foci of EMH are more common in IMF due to high volume of circulating haematopoietic growth factors and cytokines. The hypothesis behind EMH include (1) displacement of pleuripotent haematopoietic stem cells from marrow and subsequent infiltration into mesenchymal organs and (2) reactivation of embryonic rests of totipotent haematopoietic stem cells in various organs secondary to ineffective marrow haematopoeisis 6 7…”

Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis

“…At preoperative CT scan, only the latter form can be seen as a pseudo tumor whose differential diagnosis has to be made among peritoneal tuberculosis, visceral tumors and nodules of extramedullary hematopoiesis [5] , [7] , [11] , [12] , [13] .…”

Extramedullary haematopoietic tumour of the breast: a case report in a woman with secondary myelofibrosis following essential thrombocythaemia

“…However, it is also well known that the t(2;13) (q35;q14) translocation in alveolar rhabdomyosarcoma involves PAX3 on chromosome 2q35. [1][2][3] In our paper we were speculating as to why alveolar rhabdomyosarcomas should also stain with ALK protein by immunohistochemistry. We were not attempting to implicate particular genes in a definite manner, but as PAX3 is close to ATIC and the former is involved in alveolar rhabdomyosarcoma it may be the reason why ALK immunostaining is found.…”

“…Extramedullary haematopoietic tumour of the breast: a case report in a woman with secondary myelofibrosis following essential thrombocythaemia Sir: Extramedullary haematopoiesis (EH) is the ectopic production of myeloid, erythroid and megakaryocytic elements in lymphoretricular organs, usually in association with chronic myeloproliferative disorders. 1,2 It rarely occurs, as incidental findings, in non-lymphoretricular organs, 3 and even rarer is the presence of a tumour mass (extramedullary haematopoietic tumour). 4 Morphologically, these lesions may be mistaken for the more commonly encountered tumours such as carcinomas or sarcomas, especially if the clinical history is unknown.…”

“…Glandular elements are absent within the lesion, and there is no necrosis, nuclear atypia or mitotic activity. 2,3 Immunohistochemically, the tumour cells express desmin, smooth muscle actin, and vimentin, but are usually negative for S100 and cytokeratin. 4 A search of the literature failed to detect any references dealing with the oestrogen (ER) and progesterone (PgR) receptor status of these lesions, although uterine leiomyomas are known to express both receptors.…”