ABSTRACT
Sclerosing extramedullary hematopoietic tumor has been described as a rare manifestation of chronic myeloproliferative neoplasm. The lack of knowledge about this entity has caused it to be mistaken for many types of nonhematopoietic and hematopoietic tumors. We present the case of a 71-year-old lady with a long history of primary myelofibrosis, which developed multiple abdominal sclerosing extramedullary hematopoietic tumors with good clinical evolution. Nonchronic myeloid leukemia myeloproliferative neoplasm included a JAK2 mutation as part of the diagnosis algorithm. Particularly, idiopathic myelofibrosis is related with a JAK2 mutation in 50% of the cases with a pejorative prognosis. The absence of JAK2 demonstrated in the paraffin samples of the tumors may be related to the unusual evolution in this particular case. Morphologically differential diagnoses considered in the evaluation of this entity and in our case included sarcomas mainly liposarcoma, anaplastic carcinoma, and Hodgkin lymphoma.
Subject(s)
Bone Marrow/pathology , Janus Kinase 2/genetics , Primary Myelofibrosis/diagnosis , Sarcoma, Myeloid/diagnosis , Aged , Biomarkers, Tumor/metabolism , Bone Marrow/metabolism , DNA Mutational Analysis , Diagnosis, Differential , Female , Humans , Immunochemistry , Janus Kinase 2/immunology , Janus Kinase 2/metabolism , Mutation/genetics , Primary Myelofibrosis/complications , Primary Myelofibrosis/genetics , Primary Myelofibrosis/pathology , Primary Myelofibrosis/physiopathology , Prognosis , Sarcoma, Myeloid/etiology , Sarcoma, Myeloid/genetics , Sarcoma, Myeloid/pathology , Sarcoma, Myeloid/physiopathology , Sclerosis , SplenectomyABSTRACT
Agnogenic myeloid metaplasia is a hematologic disorder accompanied by extramedullary hematopoiesis (EMH) affecting various organs. Lung involvement however is rare. We present the case of a 76-year-old woman with myelofibrosis, recurrent pleural effusions, pulmonary hypertension, and serious right cardiac failure. An open lung biopsy confirmed pulmonary EMH. She underwent low-dose (200 cGy) whole-lung radiotherapy in 4 fractions of 50 cGy each. Her clinical and hemodynamic parameters improved. We conclude that low-dose whole-lung radiation may be efficacious for the palliative treatment of pulmonary EMH.
Subject(s)
Lung Diseases/diagnosis , Lung Diseases/radiotherapy , Primary Myelofibrosis/physiopathology , Aged , Biopsy , Bone Marrow/pathology , Fatal Outcome , Female , Hematopoiesis, Extramedullary , Humans , Hypertension, Pulmonary , Lung/pathology , Lung Diseases/pathology , Palliative Care , Pleural Effusion , Primary Myelofibrosis/pathologyABSTRACT
Idiopathic myelofibrosis (IMF) is a clonal chronic myeloproliferative syndrome characterized by the proliferation of the three haemopoietic series and the marrow connective tissue and by the development of extramedullary haemopoiesis in the liver, spleen and lymph nodes. Cutaneous extramedullary haemopoiesis is an uncommon event and we could not find any reported cases of testicular involvement in this disease. We report the case of a 28 year-old male with diagnosis of idiopathic myelofibrosis in November 1988. During the course of the disease, three years later, he developed a tumor on his right testis. Histologic examination showed extramedullary haemopoiesis with cells of the myeloid, erythroid and megakaryocyte series, in the interstice. Eight months later, numerous red-purple papules and nodules developed on the patients's trunk. The biopsy of a skin lesion revealed an infiltration of the dermis by myeloid, erythroid cells and few megakaryocytes. The patient's clinical condition worsened, and he died in February 1993 following progressive deterioration of the general condition. We describe a case of IFM with extramedullary hemopoiesis involving the skin and the testis pointing out the rarity of these localization.
Subject(s)
Hematopoiesis, Extramedullary , Primary Myelofibrosis/physiopathology , Skin/physiopathology , Testis/physiopathology , Adult , Humans , MaleABSTRACT
Diversos informes en la literatura han documentado alteraciones inmunológicas distintivas en pacientes con histiocitosis de células de Langerhans (HCL). Nosotros presentamos un caso de HCL en una mujer de 62 años de edad con lesión en cráneo, mielofribrosis y gamopatía policlonal asociadas a enfermedad activa. La patogenesis de esta asociación es incierta; sin embargo, hay evidencia de que esta alteración es manifestación de una pobre regulación en la activación de células de Langerhans con efecto en determinados controles inmunológicos