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At least 415 records · Page 23Linked to original sources
PubMed2002

[Treatment of chronic myelogenous leukemia with imatinib mesylate resulting in hematological remission and marked regression of myelofibrosis].

We treated two chronic phase chronic myelogenous leukemia patients with imatinib mesylate. Hematological complete remission and significant regression of bone marrow fibrosis were observed in both patients. The large amount of TGF-beta produced by increased bone marrow megakaryocytes might have caused the myelofibrosis, and it was revealed that imatinib mesylate brought about regression of the myelofibrosis by reducing the number of megakaryocytes in both patients.

Aged↗
PubMed1992

[Primary myelofibrosis successfully treated with allogeneic bone marrow transplantation].

A 31-year-old man with primary myelofibrosis initially received low dose Ara C. Splenomegaly decreased but pancytopenia continued. Allogeneic bone marrow transplantation from his sister was then performed. Busulfan and cyclophosphamide were used as a preconditioning regimen, which included neither irradiation nor splenectomy. As the bone marrow was hypoplastic after transplantation, G-CSF was given. It was useful for systemic infection. After transplantation, leukoerythroblastosis and tear drop poikilocytosis disappeared in peripheral blood. Finally, bone marrow fibrosis disappeared and hemopoiesis to normal limits recovered 17 months later. These results demonstrate that bone marrow transplantation is effective for primary myelofibrosis for which there is no otherwise curative therapy.

Adult↗
PubMed1992

[Megakaryoblastic leukemia which developed from therapy-related MDS with myelofibrosis].

A 56-year-old man had a leiomyosarcoma of the small intestine in 1987. After surgery, he received cyclophosphamide for 2 years. In December, 1990, he exhibited severe pancytopenia. His hematological data were as follows: Hb 7.4g/dl, ret. 0.8%, WBC 1,700/microliters with leukoerythroblastosis and 2.8 x 10(4)/microliters platelets. A bone marrow aspiration was a dry tap. A bone marrow biopsy specimen showed a hypercellular marrow with myelofibrosis, leukemic infiltration (10.2%) and slight dyserythropoiesis. Both PPO and GPIIb/IIIa reaction were positive for blast cells and atypical megakaryoblasts. A diagnosis of MDS with an abnormality in megakaryocytic lineage was made. The patient was treated with 1,25-dihydroxy-vitamin D3, however this therapy was temporary and he developed into acute megakaryoblastic leukemia (M7). This report suggested that some cases of therapy-related leukemia (TRL) mainly involve megakaryocytic lineage and are diagnosed as MDS with myelofibrosis which transform to M7. The fact that PAS stain of erythroblasts in the patient reported here was positive may suggest involvement of development of more precise immunological markers of differentiation and EM study will permit better diagnosis of TRL and may therefore facilitate new therapeutic approaches.

Chemotherapy, Adjuvant↗
PubMed1992

Hematopoietic progenitor cells in a patient with myeloid metaplasia without myelofibrosis.

A rare case of myeloid metaplasia without myelofibrosis is presented. Findings that the increase in blood megakaryocyte progenitor cells was not as prominent as the increase in granulocyte-monocyte and erythroid progenitor cells, and that the increase in bone marrow megakaryocytes was only slight might explain the absence of bone marrow fibrosis in this case. However, it is not clear whether the disease is a subtype or merely an early stage of myelofibrosis with myeloid metaplasia.

Aged↗
PubMedDate not supplied

Acute myelofibrosis terminating in acute lymphoblastic leukemia: report of one case.

A four-year-old boy suffered from acute myelofibrosis which became acute lymphoblastic leukemia during the course of the disease. Histologic and immunophenotypic studies revealed that the leukemic cells were lymphoblasts. Immunophenotypic examination also excluded the possibility of acute megakaryoblastic, monocytic and myeloid leukemia. Thus terminal leukemic transformation, in some cases of acute myelofibrosis, may be of a lymphoid nature.

Acute Disease↗
PubMed2005

Myeloablative conditioning in myelofibrosis using i.v. treosulfan and autologous peripheral blood progenitor cell transplantation with high doses of CD34+ cells results in hematologic responses - follow-up of three patients.

Autologous transplantation after myeloablation for myelofibrosis with myeloid metaplasia provides a palliative therapy with a long term relief of symptoms. We have transplanted three patients with more than 5 x 10(6) CD34+ cells/kg body weight after myeloablation with treosulfan (total dose 42 g/m(2)) with a 18 months follow-up. Two of the patients had symptomatic splenomegaly and severe anemia. One patient had symptomatic splenomegaly and thrombocytopenia (< 100x10(9)/L). Granulocyte colony-stimulating factor-supported peripheral blood progenitor cell mobilization and collection was not associated with increased toxicity. Following transplantation we observed a prolonged reconstitution period of 28-38 days without fever or severe mucositis. All patients became free of erythrocyte transfusions or recovered to normal thrombocyte counts. There was a significant reduction of max. spleen size in one patient. We conclude that myeloablation with treosulfan and autologous PBPCT in these three patients with myelofibrosis was safe and useful.

Antigens, CD34↗
PubMed2005

[Myelofibrosis with myeloid metaplasia: diagnosis and treatment].

Myelofibrosis with myeloid metaplasia is the rarest myeloproliferative syndrom. Diagnosis is often easy in classical form, characterised by splenomegaly, leukoerythroblastic blood reaction and tear-drop erythrocytes on blood count and myelofibrosis on bone marrow biopsy. Evolution is highly variable with a median overal survival of 40 to 60 months and numerous prognostic factors especially anemia. No treatment has been demonstrated to improve survival (apart from allogenic bone marrow transplant). The rarity and the complexity of the disease are the most important difficulties for the definition of standardized diagnostic, prognostic and therapeutic criteria.

Bone Marrow Transplantation↗
PubMed2005

[Case of intraspinal epidural tumor developing after systemic mastocytosis with marked osteosclerosis and myelofibrosis].

A 64-year-old man was diagnosed as having urticaria pigmentosa in 1998, and treated with PUVA therapy. In January 2002, X-ray imaging revealed osteosclerosis was detected in the systemic bone and bone scintigraphy. A bone marrow aspiration sample was not obtained due to a dry tap. CT scans showed hepatosplenomegaly and mesenteric lymphadenopathy. Myelofibrosis and diffuse mast cell infiltration were revealed by a bone marrow biopsy, and a diagnosis of systemic mastocytosis with severe osteosclerosis and myelofibrosis was made. In October 2003, he was admitted to our hospital because of mid back pain. A neurological examination showed muscle weakness in the upper and lower limbs, sensory disturbance below the level of Th4 and urinary obstruction. T1 and T2 weighted images of MRI demonstrated a high intensity epidural mass lesion extending from the vertebral level of C5 to Th2 and severely compressing the spinal cord. We considered the possibility of the invasion of the spinal canal by the mastocytosis. The patient was treated with interferon alpha-2b (IFN-alpha2b) and prednisolone. Subsequently, the motor and sensory disturbances were gradually alleviated, and spinal MRI confirmed a marked reduction in the size of the epidural tumor. However, the patient became resistant to interferon, and died of multiple organ failure in spite of steroid pulse and cladribine therapies. Multiple organ infiltration by mast cells was revealed at autopsy.

Antineoplastic Agents↗
PubMed2006

[Study of 161 chronic idiopathic myelofibrosis patients for clinicopathological staging].

OBJECTIVE: To investigate the significance of clinicopathological stage of chronic idiopathic myelofibrosis (CIMF) in WHO classification of 2001. METHODS: Histopathological analysis of bone marrow biopsy plastic-embedded sections stained with H-G-E and Gomori's stains and clinical features of 113 cases previously diagnosed as primary myelofibrosis (PMF) and 48 cases MPD-U (total of 161 cases which including male 79 and female 82) were studied retrospectively. RESULTS: There was no significant differences on the clinical features among the cellular phase, collagen fiber phase, sclerotic phase and osteomyelosclerosis of 113 previously diagnosed patients. According to WHO classification 2001 of CIMF, previously diagnosis in 48 cases with MPD-U was WHO pre-CIMF, and in 113 cases with PMF was WHO CIMF-Fs. There were significant differences between of WHO pre-CIMF and WHO CIMF-Fs about clinicopathological features except age. The percentage of immature granulocytes, normoblasts, lymphocytes in peripheral blood, the size of hepatosplenomegaly, and the percent age of tear drop-like red blood cells in pre-CIMF were significantly lower than those in CIMF-Fs (P < 0.05). However, the number of hemoglobin and platelets in patients with pre-CIMF were significantly higher than that with CIMF-Fs (P < 0.01). CONCLUSION: pre-CIMF and CIMF-Fs in clinical and histopathological features were different development stage of CIMF, while osteomyelosclerosis is a variant of CIMF, but not an independent disease.

Adult↗
PubMed1991

[Acute megakaryoblastic leukemia with leukemia cutis, meningeal leukemia, and myelofibrosis].

A 64-year-old man was admitted to our hospital with leukopenia. On admission, leukocyte count in the peripheral blood was 1,600/microliters, containing 24.5% blasts of lymphoid appearance, which were negative for myeloperoxidase. A bone marrow aspiration showed hypoplasia with increased blasts (31.6%). The blasts were ultrastructurally positive for platelet peroxidase (PPO) and positive for platelet membrane glycoprotein IIb/IIIa complex. A diagnosis of acute megakaryoblastic leukemia was made. Chemotherapy with behenoyl-ara C (BH-AC) (150 mg/day) was transiently effective. However, after three months, numerous nodules without itching appeared over the entire body, particularly on the anterior chest. A biopsy of the skin lesion revealed a diffuse fibrosis with infiltrations of the blasts. Bone marrow aspirations were dry tap, and a bone marrow biopsy showed marked myelofibrosis. Then, severe headache, vomiting, and loss of consciousness developed, and a lumbar puncture revealed infiltrations of blasts. Although methotrexate was intrathecally injected, he died due to the respiratory failure. As far as we know, a case of acute megakaryoblastic leukemia with leukemia cutis and meningeal leukemia is quite rare. In addition, it is interesting that megakaryoblastic leukemia was accompanied with both the fibrosis of skin and the myelofibrosis.

Humans↗
PubMed1991

[Gray platelet syndrome, an example of myelofibrosis of megakaryocytic origin].

In this study, the clinical history of two patients with the gray platelet syndrome, a rare congenital disorder associating thrombopathia and myelofibrosis is recalled. Complementary studies on platelets and megakaryocytes were performed, mainly with an immunocytochemical approach. In gray platelets, a general decrease of alpha-granule proteins, including PF4, beta tg and PDGF was observed. The decrease in platelet mitogenic activity (PDGF) was confirmed by biological and radio-immunological measurements. An abnormally high level of these compounds was also found in the plasma. In megakaryocytes cultured from the bone marrow of these patients, alpha-granule proteins were normally expressed in early maturation stages, whereas they were found to be absent in the mature megakaryocytes. An alpha-granule membrane glycoprotein, GMP 140 has been studied in resting and thrombin stimulated gray platelets and was found to be normally expressed at the surface of stimulated platelets. GMP140 was studied in resting platelets by immunoelectron microscopy and found to be present in vacuole probably corresponding to empty granules. This observation allows to conclude that alpha-granule membrane is formed in the gray platelet syndrome, but that there is a storage defect of alpha-granule soluble proteins, possibly due to an abnormal targetting of these proteins to the alpha-granule. Synthesis and subsequent release of these proteins, namely of the mitogenic factors, which can induce myelofibrosis and lung fibrosis by abnormal fibroblast stimulation, is discussed.

Adult↗
PubMed1991

[Idiopathic myelofibrosis with cutaneous extramedullary hematopoiesis].

Idiopathic myelofibrosis is a chronic myeloproliferative syndrome characterized by the proliferation of the three hemopoietic series and the marrow connective tissue and the development of extramedullary hemopoiesis in the liver and spleen. Cutaneous extramedullary hemopoiesis is and uncommon finding, and only thirteen cases have been reported up to now. We report the first case in the Spanish literature. The patient was a 78-year-old male in whom skin lesions preceded the diagnosis of myelofibrosis. These lesions, consisting of violet coloured maculae and papulae, contained elements from the three hemopoietic series. Therapy with hydroxyurea was started, but the patient died because of cardiorespiratory complications.

Aged↗
PubMedDate not supplied

[Idiopathic myelofibrosis].

The authors make a review of recent data in the literature and compare them to their own cases between 1983 and 1988. They analyzed eight patients with idiopathic myelofibrosis confirmed with bone marrow biopsy of the posterior iliac wrist with Jamshidi's needle. Most patients were between 55 and 60 years old (5 male and 3 female). Major symptoms were weakness and bleeding (6/8 cases), weight loss and bone distress (4/8 cases). In general, physical signs were splenomegaly and anemia (7/8 cases), hepatomegaly (5/8 cases), and jaundice (2/8 cases). Laboratory features were variable. Most cases were diagnosed in an advanced stage of the disease. Therapy with busulfan, prednisone, oxymetholone and radiotherapy of the spleen was used alone or in combination to relieve compressive abdominal symptoms. This review shows that idiopathic myelofibrosis should be included in the differential diagnosis of patients with hepatosplenomegaly and anemia.

Adult↗
PubMed1991

Ultrastructure of bone marrow tissue in so-called primary (idiopathic) myelofibrosis-osteomyelosclerosis (agnogenic myeloid metaplasia). II. The myeloid stroma (hematopoietic microenvironment).

An ultrastructural study was performed on bone marrow tissue in 8 patients revealing early and late stages of so-called primary (idiopathic) myelofibrosis - osteomyelosclerosis (agnogenic myeloid metaplasia) to evaluate the constituents of the hematopoietic microenvironment (myeloid stroma). A survey of the stroma cells disclosed an overall increase, particularly in so-called undifferentiated (primitive - pluripotent), but also in transitional (fibroblastic) reticular cells and myofibroblasts. The most primitive reticular cells were characterized by their stellate aspect with elongated slender cytoplasmic processes traversing the interstitial space, and by the scarcity of organelles. The transition into a fibroblast was preceded by the appearance of branching cisternal structures of the rough endoplasmic reticulum, extensively developed Golgi fields and an abundance in mitochondria. Frequently, so-called myofibroblasts were encountered displaying bundles of filaments along the subplasmalemmal region. Extracellularly fibrillar material with an irregular cross-banding as well as microfibrils could be observed. The many vascular structures (sinusoids and capillaries) exhibited a multilayered basement membrane-like material including many fibrils and adventitial cells (pericytes, smooth muscle and transitional reticular cells) with numerous cytoplasmic processes. Undifferentiated and transitional reticular cells as well as myofibroblasts seem to form an integral part of the hematopoietic microenvironment in OMF and are assumed to play an important role for the evolution of the disease-specific myelofibrosis in this disorder.

Bone Marrow↗
PubMed1991

Ultrastructure of bone marrow tissue in so-called primary (idiopathic) myelofibrosis-osteomyelosclerosis (agnogenic myeloid metaplasia). I. Abnormalities of megakaryopoiesis and thrombocytes.

An electron microscopic study was conducted with the aim to analyze abnormalities of the megakaryopoiesis in bone marrow tissue of patients suffering from so-called primary (idiopathic) myelofibrosis-osteomyelosclerosis. In comparison with control specimens and in confirmation of light microscopical findings, a pronounced pleomorphism of the megakaryocytic cell line could be observed consisting of giant forms, micromegakaryocytes and naked (pyknotic) nuclei. Further atypias were expressed by a dissociation of nuclear-cytoplasmic maturation. These included particularly the amount of dense granules, and the development of the demarcation membrane system as well as the occurrence of emperipolesis (i.e. internalization of hematopoietic cells) already in immature or megakaryoblastic elements. In these specimens there was a striking variety in the appearance of dense granules of the alpha or bull's eye type revealing frequent elongated roll- and dumbbell-like shapes and a doubling of the nucleoids. Thrombocytes showed giant forms with either hypertrophy of the open canalicular system or abundance of dense granules and beta-glycogen accumulation. Remarkable was a focal sponge-like proliferation of the open canalicular system in many of the large platelets and giant and fused granules of the alpha and osmiophilic type. The abnormalities disclosed in megakaryo- and thrombocytes may have certain functional implications, i.e. to hemorrhage and thrombosis which are often encountered out of proportion to the platelet counts in this disorder. Moreover, those anomalies indicate a disorganization of megakaryopoiesis which may contribute to the abnormal release of factors (platelet-derived growth factor and factor 4) predominantly involved in the process of myelofibrosis.

Blood Platelets↗
PubMedDate not supplied

Acute myelofibrosis--a leukemia of pluripotent stem cell. A report of three cases and review of the literature.

The histogenesis of blasts in acute myelofibrosis is generally regarded to be of megakaryocytic origin. Three case reports are presented and 19 other reported cases were reviewed from the literature where the cells of origin appear to be myeloblasts, myelomonoblasts, lymphoblasts, or undifferentiated blasts. It is therefore postulated that acute myelofibrosis is a hemopoietic stem cell disorder, and acute megakaryocytic leukemia (FAB-M7) represents one subset of the disorder.

Aged↗
PubMed1990

[Beta-thromboglobulin and platelet factor 4 in a follow-up of acute myelofibrosis (megakaryoblastic leukemia)].

We report a patient with acute myelofibrosis (AM) in whom a megakaryocytic origin was demonstrated after conventional microscopy, investigation with monoclonal antibodies directed against the glycoprotein complex IIb/IIIa (CD41a) and the platelet peroxidase (PPO) reaction. Thus, a diagnosis of acute megakaryoblastic leukemia (AMGL) was made. It is now known that this megakaryoblastic proliferation is responsible for myelofibrosis as an increased release of platelet-derived growth factor (PDGF), beta-thromboglobulin (BTG), and platelet factor 4 (PF4) develops because ineffective megakaryocytopoiesis and failure of these clonal populations to store the mentioned substances in their alpha granules. At the time of diagnosis, the plasma concentrations of BTG and PF4 were measured and were found to be high. Thus, an increased PDGF level was indirectly assumed, with the subsequent fibroblast stimulation. After treatment with low dose cytosine arabinoside, a clinical, analytical and histological remission was achieved, with a return of BTG and PF4 values to the normal range. It was therefore concluded that the follow up of these parameters is useful for the diagnosis and the establishment of remission criteria in these patients.

Acute Disease↗