Idiopathic myelofibrosis with nodal, serosal and parenchymatous infiltration. Case report and review of the literature.
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.
Tzankov
Alexander
A
Institute of Pathology, University of Innsbruck, Austria. alexander.tzankov@uibk.ac.at
Krugmann
Jens
J
Steurer
Michael
M
Dirnhofer
Stephan
S
D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006411:Hematopoiesis, Extramedullary; D006412:Hematopoietic Stem Cells / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology*; D008206:Lymphatic Diseases / Q000473:pathology; D008533:Megakaryocytes / Q000473:pathology; D010534:Peritoneal Neoplasms / Q000473:pathology; D010537:Peritoneum / Q000473:pathology*; D010976:Platelet Count; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000473:pathology*; D012186:Retroperitoneal Neoplasms / Q000473:pathology; D013154:Spleen / Q000473:pathology; D014057:Tomography, X-Ray Computed