SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
An 11-year-old boy developed primary myelofibrosis. Nine months after diagnosis he received high-dose chemoradiotherapy followed by T cell-depleted allogeneic bone marrow transplantation as primary therapy. Sixteen months after grafting the patient is well, with reversal of the marrow sclerosis and no evidence of chronic graft-versus-host disease.
A retrospective study was conducted on 14 patients with idiopathic myelofibrosis after splenic irradiation in order to cure the symptoms due to secondary splenomegaly. Radiation dose ranged from 700 to 2400 cGy, depending on both clinical response and hematological toxicity. Relief of symptoms was observed in all patients (100%); reduction of splenic size ranged from 30 to 70%, with more than 50% reduction in 13/16 cases. Side effects were mild neutropenia and thrombocytopenia, which did not require interruption of treatment; four patients registered a severe anemia. All the hematological side effects stopped with the suspension of treatment. This study shows the beneficial effect of radiation therapy on the symptoms connected to splenomegaly; however, the short duration of response suggests the advantage of splenic low-dose irradiation, to be periodically repeated.
The Authors present a case of acute myeloblastic leukemia secondary to a busulfan and splenic radiotherapy treated idiopathic myelofibrosis. The presence of D group complex chromosomal abnormalities could be responsible for the simultaneous massive Pelger anomaly of granulocytes.
A search for immunoglobulin deposits in normal and fibrous interstitial tissue was carried out on 100 bone marrow biopsies (44 myeloproliferative disorders and 56 other hemopathies). This preliminary study enabled one to draw three conslusions: 1) The search for immunoglobulins is only possible on non-decalcified bone marrow. The direct immunofluorescent technique gives better results than peroxidase staining: 2) In myeloproliferative disorders immunoglobulins (especially G) are more frequent and more often associated with a myelofibrosis than in other hemopathies, and 3) The more pronounced the fibrosis the more frequent the immunoglobulin deposits, except at the osteomyelosclerosis stage. They are never found in the fibrosis secondary to intramedullary carcinomatous metastases.
A 62-year-old woman with myelofibrosis developed bluish infiltrations on the trunk. Histologically, the lesions showed extramedullary hematopoiesis consisting of myeloid, erythroid, and megakaryocytic elements.
Studies have been carried out with cultured human megakaryocytes derived from Gray Platelet Syndrome blood and have demonstrated that MK-DGF and MK-F4 are present in the early stages of megakaryocyte maturation (day 5-6) but disappear in the late phase (day 9-11). In Gray platelets total mitogenic activity, PDGF and PF4 amounts were reduced while beta TG and PF4 plasma levels were slightly increased. These findings indicate a possible relationship between the loss of alpha granule contents from the megakaryocytes in the marrow and the myelofibrosis found in Gray platelet disorder and possibly associated in other diseases with platelet or megakaryocyte abnormalities.
Cutaneous hematopoiesis occurring in adult life is a rare but specific manifestation of myelofibrosis. We report a case where the three components, i.e., myeloid, erythroid and megakaryocytic cells, were present in the dermis.
Mastocytosis is an unusual disorder of unknown etiology characterized by an abnormal proliferation of tissue mast cells. We present a case of a young woman with systemic mastocytosis with malabsorption secondary to small bowel invasion by mast cells, myelofibrosis, and massive exudative ascites secondary to mass cell invasion of the intraabdominal lymph nodes. The patient responded favorably to a combined therapy with histamine H1 and H2 receptor antagonists and corticosteroids. We include a review of the literature.
A 42-year-old man with severe pancytopenia and myelofibrosis underwent splenectomy seven months after onset of his symptoms; the leukocyte, platelet, and hematocrit levels became normal. Myeloid metaplasia was identified in the liver and spleen. Progressive lymphocytosis started eight months after splenectomy, and after 66 months a florid hairy-cell leukemia was diagnosed; the circulating cells were B type with micro K surface markers. Anemia and thrombocytopenia reappeared and were controlled initially with daily prednisone; chlorambucil was later added. At that time, the peripheral blood had more than 150 megaloblastoid-appearing normoblasts per 100 leukocytes. The PAS stain was positive in 95% to 100% of these cells; the B-cell surface markers were no longer identified. Further treatment failed to control the lymphoproliferative and myeloproliferative syndromes; the patient died 99 months after splenectomy. On autopsy, infiltration by hairy-cell leukemia cells and erythroid precursors was observed in the bone marrow, liver, lymph nodes, and other organs.
We undertook a study of 35 cases of myelofibrosis with myeloid metaplasia to assess the histopathologic findings in the bone marrow of patients with this disorder, to ascertain if changes in morphology occurred with time, and to attempt to correlate marrow findings with splenic size. We reviewed 71 bone marrow biopsies and studied 13 splenectomy specimens. Sequential bone marrow biopsies were obtained in 21 cases over intervals ranging from two to ten years (mean, 4 1/2 years). We noted a patchy nature and variable degree of stromal proliferation in most marrow biopsies, and were unable to demonstrate a correlation between the extent of medullary fibrosis and duration of disease, splenic weight, or degree of splenic myeloid metaplasia. We were unable to document a progression of medullary fibrosis as a cause for increasing splenomegaly. However, the alteration in the marrow stroma in this disorder is responsible for the presence of distended marrow sinusoids with intravascular hematopoiesis, a phenomenon we observed in all cases. We believe that this morphological feature, not emphasized by previous investigators, is of significance in understanding the pathophysiology of myeloid metaplasia.
Two patients with IgD myeloma protein and lambda light chains are described. They had marked Bence Jones proteinuria, were treated with melphalan, prednisone, and allopurinol and died in less than one year. At autopsy, there was extensive myelofibrosis associated with neoplastic infiltrates of plasma cells in both cases. On electrophoresis, the serum and urine Bence Jones protein of one patient migrated in the alpha2 region, and the blood vessels of the other patient contained many nodular deposits of amyloid. Because these findings are rare in multiple myeloma, they may be unique to IgD myeloma.
Ten cases of Hodgkin's disease was massive bone marrow invasion. This particular form of the disease is seen either at the outset or in Stage IIIB with a rapid course. It is reflected by a pancytopaenia with poor marrow with little or no superficial nodes. These very atypical forms of Hodgkin's disease must thus always be considered as a possibility in the context of acute malignant myelofibrosis. They indicate a special and unexplained behaviour of the host to the Hodgkin's tumour.
A 42-year-old patient with a regenerative erythroblastopenic anaemia (7 g % Hb), considered as Pure Red Cell Aplasia (PRCA) is submitted to an immunosuppressive treatment (cyclophosphamide + prednisone). For three times, this treatment gives a complete remission, followed by relapse when medication stopped. Hereafter the patient develops a Myelofibrosis with Myeloid Metaplasia (MMM). Between the different hypothesis, the most probable one seems to be an autoimmune PRCA secondary to MMM but appearing before the latter. This observation allows discussion about the role of immunological phenomens in MMM.
The pathological and ultrastructural features of the bone marrow and the kinetics of megakaryopoiesis in patients with primary myelofibrosis indicate that a vast number of developing megakaryocytes die within the bone marrow (ineffective megakaryopoiesis). This leads to an excessive concentration of megakaryocyte intracytoplasmic components in the marrow intercellular space. Current concepts of marrow collagen regulation and megakaryocyte composition lend support to the hypothesis advocating that the development of marrow fibrosis involves two megakaryocyte components: growth factor and factor 4. The growth factor stimulates fibroblast proliferation and collagen secretion while factor 4 inhibits the activity of the enzyme collagenase. The imbalance between increased collagen production and decreased collagen degradation results in an excessive deposition of collagens in bone marrow matrix.
The Authors, after some theoretical considerations on cell Kinetics, report five cases of Primary Asplenomegalic Myelofibrosis. Pointing out some data about the diagnosis, the Authors take into consideration the clinical course of the disease which, according to the literature, appears significantly more severe.
The caryotype of a patient't leukemic cells with primary myelofibrosis being at the stage of blast crisis is described. In all cells examined a great submetacentric chromosome was detected which was formed by the duplication of the whole long arm of the first chromosome. The second chromosome of the first pair remained unchanged. Moreover, there was no sexual chromosome Y in seven metaphase plates. The significance of these and other changes of the first chromosome in the genesis of some malign diseases is discussed.
A case of hereditary autosomal recessive thrombocytopenia is reported. Thrombocytopathy is associated with the thrombocytopenia. There is a contrast between the cytologic aspect of poor bone-marrow without any megakaryocyte and the histologic aspect of dense bone-marrow with a normal number of megakaryocytes. Myelofibrosis can explain this discrepancy. The life time of platelets being just a little shortened, the disease is probably due to a lack of bone-marrow production.
Acute myelofibrosis (AM) or malignant myelosclerosis is a myeloprofilerative syndrome in which bone marrow fibrosis is associated with a proliferation of immature myeloid cells. In four patients with typical AM, investigated by the platelet-peroxidase reaction at ultrastructural level, the blast cells were found to be megakaryoblasts. One patient, treated with the drug combination DAT, achieved a complete remission of 5 mo duration. This study supports the view that megakaryoblastic leukemia is the most frequent underlying cause of AM and proposes that it should be classified as a form of acute myeloid leukemia.