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PubMedDate not supplied

Primary myelofibrosis terminating in megakaryoblastic crisis.

Primary myelofibrosis terminating in megakaryoblastic crisis is uncommon. A case with this condition is reported. The patient, a 62-year-old female, having had primary myelofibrosis for 13 years and a splenectomy 4 years before, was admitted because of high fever, hepatomegaly, thrombocythemia and leukocytosis. On admission, blasts appeared in the peripheral blood and thereafter gradually increased in number. The blasts were proven to be of megakaryocytic lineage. To our knowledge this is the third case of primary myelofibrosis terminating in megakaryoblastic crisis to be reported in Japan.

Female↗

PubMedDate not supplied

[Autoimmunity and deficiency of cell-mediated immunity in primary myelofibrosis].

Untreated patients with primary myelofibrosis show (1) a high frequency of cutaneous anergy, (2) normal lymphocytosis with decreased level of E-rosette-forming cells in most patients, (3) normal lymphocyte reactivity to PHA and in MLR, and (4) a relative frequency of LE cells and anti-organ antibodies.

Autoimmune Diseases↗

PubMed1977

Clinical course and survival after elective splenectomy in 19 patients with primary myelofibrosis.

Nineteen patients with primary myelofibrosis and myeloid metaplasia (MMM), who fulfilled well-defined criteria, underwent elective splenectomy as soon as the diagnosis was confirmed. Nine patients developed postoperative complications such as intra-abdominal bleeding (three), rupture of the inferior vena cava (one), ascites (two), Australia antigen-positive hepatitis (three), mesenteric artery thrombosis (one) and pneumonia (one). One patient died within a month of the operation due to secondary intra-abdominal infection. The mean age of the patients at splenectomy was 56 years and the mean duration of their disease 2.4 years. The median actuarial survival after operation was 51 months. Although the series of patients is small, it seems that splenectomy did not have an adverse effect on life expectancy. The haematological status and the quality of life improved after splenectomy in 17 of 19 patients. The results warrant a further trial with elective splenectomy in an early stage of MMM.

Adult↗

PubMedDate not supplied

[Successful treatment of primary myelofibrosis with thrombocytosis during pregnancy with alfa-interferon].

Primary myelofibrosis is predominantly a disease of old age, poor prognosis and no curable treatment. Thrombocytosis was observed in only 12% of patients. To our knowledge, there is only one reported case of a young woman with primary myelofibrosis who had a term pregnancy [1]. We report on a 29-year-old woman with thrombocytosis and medical history of two miscarriages in the last 2 years, the iirst at 30 weeks of gestation and the second at 27 weeks. Multiple placental infarctions were observed. She was without symptoms but with moderate splenomegaly 4.5 cm below left costal margin). The platelet count was 651 x 10(9)/L, WBC 7.2 x 10(9)/L with normal differential formula, and haemoglobin level 12 g/dl. Bone marrow biopsy showed fibrotic phase of primary myelofibrosis, with hyperplasia of megacaryocytes, decreased numbers of erythroid and granulocytic cells, and increased amounts of reticulin fibres. Cyctogenetic examination of the bone marrow showed normal female caryotype. Increased numbers of progenitors CFU-Mk, CFU-GM and BFU-E were observed in peripheral blood, and decreased numbers in bone marrow cultures. As the patient wished to become pregnant, the treatment with interferon-a (Roferon A) was started at a dose of 3 MU s.c., three times per week. The platelet count rapidly decreased at a level of 260-370 x 10(9)/L. The pregnancy was diagnosed 5 months later. At the 24 week of pregnancy, platelet count raised to 690 x 10(9)/l and the dose of interferon-a was augmented, 3 MU every day, until delivery. Foetal growth and placental circulation were monitored by serial ultrasonography. At the end of 34 weeks of pregnancy, it was noted that placental flow became insufficient, and after foetal lung maturity was stimulated with dexamethasone, Cesarean section was performed. Male baby was born, weighting 2000 g, with respiratory distress syndrome. This complication was successfully treated, and the child is now one year old, with normal growth and development. The mother is still on therapy with interferon-a, 3 MU, three times a week, and the last blood count was as follows: haemoglobin 10.7 g/dl, WBC 6.1 x 10(9)/L and platelet comt 437 x 10(9)/L. In conclusion, according to the clinical results of interferon-d in thrombocytosis and experimental studies which showed the absence of placental transfer of interferon-d, this therapy could be recommended to women with primary myelofibrosis who wish to have a baby.

Adult↗

PubMed1979

[Clinical characteristics of primary myelofibrosis].

The authors followed-up five patients with primary myelofibrosis in the course of 4 years. The diagnosis, in all cases, was confirmed via histologic investigation of trepenation biopsy material of bone marrow and bone. The clinical and hematologic variety, observed by the authors, provided grounds for the formation of the following variants of the primary myelofibrosis: myelemic-anemic form, form with leucosis transformation, form with sarcomatization, myelofibrosis with immune hemolytic anemia and myelofibrosis in aplastic state. The establishment of transitory forms between the myeloproliferation and dysanaplastic blast processes allows the presumption that the pathogenesis essence of primary myelofibrosis is manifested in clonal lesion of the stem bone-marrow cells.

Aged↗

PubMed1997

Allogeneic marrow transplantation for primary myelofibrosis and myelofibrosis secondary to polycythaemia vera or essential thrombocytosis.

Primary myelofibrosis is a clonal haemopoietic disorder, incurable with conventional therapy, and associated with a median survival of 4-5 years. Patients with polycythaemia vera and essential thrombocytosis who progress into a myelofibrotic picture also have a poor prognosis. Between 1980 and 1996, 13 patients with myelofibrosis due to one of these three myeloproliferative disorders (primary myelofibrosis [n=8], essential thrombocytosis [n=3], polycythaemia vera [n=2]) underwent allogeneic marrow transplantation in Seattle. The median age was 40 years (range 18-49). The median time from myeloproliferative diagnosis to transplantation was 39 months (range 5-192). Three patients received preparative regimens containing total body irradiation and 10 received busulphan-cyclophosphamide regimens. Nine patients received marrow from HLA-matched related donors, one from a one antigen mismatched related donor, and three from HLA-matched unrelated donors. The median time to both granulocyte and platelet engraftment was 21 d. Nine patients survive between 1.2 and 7.1 years post-transplant. Two patients relapsed at 1 year post-transplant, both of whom survive in a chronic myeloproliferative state. Four patients died of transplant-related complications between 43 d and 2.2 years post-transplant. At 1 year post-transplant the majority of the disease-free survivors have normal peripheral blood counts and none-to-minimal marrow fibrosis. These preliminary results are encouraging, and suggest that stem cell transplantation can be curative therapy for selected patients with myelofibrosis.

Adolescent↗

PubMed1991

Defective erythropoiesis in primary myelofibrosis associated with a chromosome 11 abnormality.

A case of primary myelofibrosis was identified with a previously unreported complex karyotype with two abnormal clones in addition to a proportion of normal cells: 46,XY,-2,-11, + der(2)t(2;11) (q24/31;q13), + mar and 45,XY,-2,-11, + der(2)t(2;11)(q24/31;q13), + mar, -17, del(7q). Study of circulating committed progenitors from this patient consistently showed (1) an absence of erythroid progenitors which is uncommon and (2) greatly increased granulocyte-monocyte progenitors (CFU-GM) which is generally observed in myelofibrosis. Further study showed that peripheral blood mononuclear cells co-cultured with irradiated normal bone marrow stroma generated increased numbers of CFU-GM compared with controls but failed to generate erythroid progenitors, providing evidence for an intrinsic defect in erythropoiesis. Only once previously has the absence of erythroid progenitors in primary myelofibrosis been studied in relation to cytogenetic abnormalities. This case also revealed a complex karyotype which, however, shared with our case a defect on chromosome 11. The identification of two cases of primary myelofibrosis which lack committed erythroid progenitor cells and which show in common a chromosomal defect on chromosome 11 point to the existence of genes on this chromosome which play a key role during erythropoiesis.

Aged↗

PubMed1996

[Primary myelofibrosis with positive coombs' test responding to prednisolone].

Primary myelofibrosis was diagnosed in a 43-year-old female in 1991, who gradually became transfusion dependent. There was no evidence suggesting connective tissue diseases except for biological false positive STS. Because her direct Coombs' test was positive and serum haptoglobin level was extremely low (< 6mg/dl), intermediate dose (30mg/day) prednisolone therapy was started. Her hemoglobin level and platelet count increased dramatically to a normal level within 3 weeks after the initiation of prednisolone therapy. Bone marrow biopsy performed 6 months later revealed marked recovery of hematopoiesis. Though the effects of corticosteroid therapy in primary myelofibrosis still remain unclear, this therapy might be useful in the treatment of some groups of patients via the correction of immunological aberrations and/or the decrease of bone marrow fiber.

Adult↗

PubMed1976

Colony forming activity, density distribution and morphological features of the stem cell population in primary myelofibrosis.

In four patients with untreated primary myelofibrosis, the peripheral-blood cell growth in agar culture (frequency of clusters and colonies) was studied. As cell sources, were used either the buffy coat or the fractions obtained from an albumin discontinuous density gradient, prepared according to Dicke's technique. Besides the agar culture, also morphological features of the blasts of the granulocytic cell series of the gradient fractions were investigated, in order to relate the above-mentioned cell parameters. The results, taken as a whole, pointed out: a) increase in circulating CFUc's (expressed as the number/10(5) nucleated cells) in the patients, when compared to the controls; b) high percentage of CFUc's with an abnormal specific density (below 1.062 in 3 out of 4 patients); c) presence in 3 out of 4 patients, of two populations of blasts: one showing low specific density, small size, high N/C rate, high plating efficiency and no nucleoli; the other with a higher specific density and low plating efficiency, showing the typical morphological features of the myeloblasts.

Adult↗

PubMed1989

[The use of MCNU to a patient of primary myelofibrosis complicated with pericardial effusion and proteinuria].

A case of primary myelofibrosis complicated with pericardial effusion and proteinuria is described. A 66-year-old female was admitted to our hospital because of abdominal fullness and shortness of breath. On admission, hepatosplenomegaly and pericardial effusion were observed. Blood examination revealed leukoerythroblastic anemia and thrombocytosis with tear drop cells and giant platelets. Bone marrow aspiration was dry tap and its biopsy showed remarkable myelofibrosis. Urinalysis indicated severe proteinuria. Although neutrophilic alkaline phosphatase score was low, no signs of acute blastic crisis of chronic myelogenous leukemia was found. The diagnosis of an atypical type of primary myelofibrosis was obtained. Administration of MCNU was started in August 1987. Hepatosplenomegaly, pericardial effusion and proteinuria were gradually improved after the administration. The etiology of the pericardial effusion and proteinuria were not obvious, however, these facts suggest that these abnormal findings might be related to PMF itself and MCNU was effective to PNF.

Aged↗

PubMed1990

[Blastic crisis of primary myelofibrosis associated with multiple myeloblastomas ].

The patient is a 71-year-old female who underwent splenectomy due to splenomegaly 32 months after diagnosed as having primary myelofibrosis. On examination she was found to have massive skin nodules, lymph nodes swelling and an enlarged liver with an abnormal hematologic profile as follows: RBC count 3.68 x 10(6)/microliters; WBC count 151 x 10(3)/microliters with 11% blasts; and platelet count 42 x 10(3)/microliters. The bone marrow aspirate showed a hypocellular marrow with 19.2% blasts. Histological examination of the skin nodules revealed that they were myeloblastomas, thus suggesting leukemic transformation of primary myelofibrosis. Her WBC count dropped to about 20 x 10(3)/microliters through treatment with vindesine, cyclophosphamide, 6-mercaptopurine and prednisolone, but it did not drop further. Treatment with dexamethasone remarkably regressed the myeloblastomas, but she died of heart failure 4 months after diagnosis of leukemic transformation of primary myelofibrosis. The autopsy findings showed the formation of numbers of myeloblastomas in both the systemic fatty tissue and dura mater as well as extramedullary hematopoiesis in liver and lymph nodes. A rapid development of splenomegaly in a patient with primary myelofibrosis seems to be associated with leukemic transformation.

Aged↗

PubMed1985

Stromal cells in primary myelofibrosis: ultrastructural observations.

The bone marrows of five patients with primary myelofibrosis at different stages of the disease have been studied. In the myelofibrotic bone marrow, associated with "reticulum cells", two other cell types have been identified, namely fibroblast-like and myofibroblast-like reticulum cells, as well as a spectrum of transitional forms. Our findings suggest that reticulum cells may represent a reserve stromal cell pool (i.e. primitive reticulum cells) able to modulate themselves and to transform differently according to functional requirements. Some suggestions regarding the functional significance of fibroblast-like and myofibroblast-like reticulum cells in primary myelofibrosis are suggested.

Biopsy↗

PubMed1990

[Successful high-dose methylprednisolone therapy in a patient with primary myelofibrosis].

We are presenting a patient with primary myelofibrosis who responded to the High-Dose methylprednisolone therapy (1 g/day for 3 days). Three and a half years ago, a 55-year-old male was admitted to our hospital because of severe erythroblastic anemia, thrombocytopenia, splenomegaly and "dry tap" of bone marrow aspiration. Bone marrow biopsy revealed a marked fibrosis without any blastoid cell proliferations. Since a thrombocytopenia was progressive and refractory to the ordinary therapy, high-dose methylprednisolone therapy was performed which was followed by an administration of activated vitamin D3. After the therapy, hematologic improvements were achieved within a month (RBC: 284 x 10(4)/microliters----413 x 10(4)/microliters, WBC: 3,000/microliters----11,500/microliters, Plat.: 7,000/microliters----20,000/microliters). Three months after the therapy, the bone marrow biopsy and 113In scintigraphy were performed. These tests also proved marked improvement of histological features of the bone marrow and a decrease of uptake of 113In to the spleen, respectively. The patient continues to be in good condition and he is free from any medications at present time.

Bone Marrow↗

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↗

PubMed1995

[Transient promyelocytic expansion in primary myelofibrosis].

A 46-year-old man with primary myelofibrosis developed polyarthralgia. Marked hepatosplenomegaly was noted, and hematological examinations revealed a white cell count of 25,600/microliters with 42% promyelocytes and thrombocytopenia. The promyelocytes were positive for CD4 antigen and nonspecific esterase as well as peroxidase. Cytogenetic analysis of circulating mononuclear cells showed the trisomy of No. 22 chromosome in 3 of 5 cells examined. Four months later, the patient became asymptomatic, and hematological picture and hepatosplenomegaly returned to the original level. This is the first report describing the transient promyelocytic expansion in myeloproliferative disorders.

Chromosomes, Human, Pair 22↗

PubMed1990

[Primary myelofibrosis with extramedullary haematopoiesis of the skin transformed into acute megakaryoblastic leukemia].

A 70-year-old man, who had been diagnosed as primary myelofibrosis in 1987 at Tokyo Women's Medical School, was admitted to our hospital because of left hip-joint pain in May 1988. Physical examinations revealed marked hepatosplenomegaly and multiple reddish papules on the skin. The peripheral blood showed marked leukoerythroblastosis and severe anemia with poikilocytosis. Bone marrow aspirations were dry tap. Needle biopsy of iliac crest showed a diffuse fibrosis. Biopsy of the papules showed an extramedullary haematopoiesis. He was treated with low dose Ara-C for seven days. Although hepatosplenomegaly and skin papules were reduced, he died of pneumonia a month later. At autopsy, leukemic cells massively infiltrated into the multiple organs, including bone marrow. The cells were identified with megakaryoblast, as those were positive for factor VIII related antigen. As far as we know, this is the third case of primary myelofibrosis transformed into acute megakaryoblastic leukemia.

Aged↗

PubMed1995

[Transformation into chronic myelomonocytic leukemia in a patient with primary myelofibrosis associated with severe hypoplasia: report of an autopsy case].

A 70-year-old male was admitted because of anemia in September 1989, and primary myelofibrosis was diagnosed based on the presence of leukoerythroblastosis, a normal chromosomal analysis and pathological findings of fibrosis in bone marrow. Although he was anemic, he did not require any treatment for two years. Then his hematological status deteriorated to severe pancytopenia, and the marrow biopsy revealed marked hypoplasia with fatty replacement and scattered fibrosis. He was treated with metenolon without success and frequent transfusion of packed red cell was required. This hypoplastic status continued for seven months. In May 1992 his WBC count increased gradually with monocytosis. The marrow was filled with various stages of monocytes, with almost no fibrosis remaining. The chromosomal analysis was repeated but disclosed no abnormalities, consistent with the negative result of BCR-ABL rearrangement investigated by the RT-PCR method. One month later, when the patient died of multiple cerebral bleeding and infection, the leukocyte count exceed 90,000/microliters. It is known that major causes of death for patients with primary myelofibrosis are infection, bleeding, cardiac trouble and transformation to leukemia. We describe a case of myelofibrosis who developed to chronic myelomonocytic leukemia following severe aplastic phase.

Aged↗

PubMed1989

Cytogenetic and molecular studies in primary myelofibrosis.

Cytogenetic and molecular data of three patients affected by primary myelofibrosis with myeloid metaplasia (PMMM) evolving to blastic crisis are reported. The cytogenetic findings were uncommon. The first patient (female) showed an idic(X)(q13) as the sole alteration in chronic phase, with an additional r(7) in 67% of the cells of the blast crisis; the other two patients showed, in blast crisis, a partial trisomy of the long arm of chromosome 1, without translocation, as a unique structural abnormality. These findings confirm the presence of nonrandom, although nonspecific, alterations in PMMM that, in our cases, seem to be related to the multistep progression of the neoplastic process. Molecular investigations have been applied to study the genomic organization and the level of expression of genes such as bcr and calcyclin and c-fms protooncogene possibly involved in the molecular mechanisms underlying cell proliferation in hematopoietic cells. The data obtained are discussed with respect to the myeloproliferative disorder.

Aged↗