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PubMed1978

Myelofibrosis associated with multiple myeloma.

Two patients had both multiple myeloma and myelofibrosis. One, who had both conditions, had autopsy confirmation of diagnosis three months later. The typical picture of agnogenic myeloid metasplasia with myelofibrosis developed in the second patient almost four years after the onset of k light chain myeloma. At this time all evidence of multiple myeloma had disappeared perhaps related to cyclophosphamide therapy. We were able to find two similar cases described in the English language literature. The two conditions may be related.

Aged↗
PubMed1984

Effect of alkylating agents on hematopoiesis in myelofibrosis. 4 case report.

Four patients presenting with myelofibrosis (2 primary myelofibrosis and 2 postpolycythemic myeloid metaplasia) were treated with alkylating agents. For three patients (one treated with busulfan and two treated with chlorambucil) the treatment was a success: the general condition improved, the splenomegaly decreased or disappeared, and the blood picture returned to normal. Moreover, for two cases, a trend towards polycythemia was observed under treatment. For the fourth patient, treated with chlorambucil, there was no improvement: a life-threatening, pancytopenic phase developed at the end of the treatment, but it disappeared after 2 months. For the three successfully treated cases, a redistribution of hematopoiesis from spleen to bone marrow was shown by ferrokinetics, 59Fe scans, and bone marrow biopsies. In addition, in the case treated with busulfan, a decrease in the bone marrow granulopoietic pool at the expense of the erythropoietic one was observed. No redistribution was seen in the patient for whom the treatment was a failure. In this case, the spleen remained the major site of active hematopoiesis. Studies on blood granulomonocytic-colony forming cells (GM-CFC's) helped to discriminate the successfully treated patients from the unsuccessfully treated one. In the successfully treated patients, the GM-CFC concentrations dropped to normal values and increased again within weeks following the treatment interruption; this increase involved mainly high density GM-CFC's (greater than 1.060). In the unsuccessfully treated patient, GM-CFC concentrations decreased only after 5 weeks of intensive treatment. The mean density of the GM-CFC's was 1.064 before treatment, shifted towards 1.060 during the neutropenic phase and returned to 1.064 during the recovery.

Alkylating Agents↗
PubMed1983

Progressive myelofibrosis in agnogenic myeloid metaplasia.

Progression of myelofibrosis in agnogenic myeloid metaplasia (AMM) has only rarely been documented because of the paucity of patients with sequential bone marrow examinations. At our institution, 27 patients with AMM underwent marrow examinations, separated by at least six months. Of the 20 patients who did not have maximal myelofibrosis shown by the original biopsy, 18 displayed temporal progression of the process; 11 of them had an increase of two or more grades using the Bauermeister scale. The grade remained constant in the remaining two patients. Several cases of a decreased degree of fibrosis or variation of grade in concomitant specimens showed that fibrosis may not proceed at an identical rate throughout the marrow and that the degree may vary with location.

Adult↗
PubMed1981

Immunologic abnormalities in myelofibrosis with activation of the complement system.

Eighteen patients with agnogenic myeloid metaplasia with myelofibrosis were studied for clinical and laboratory evidence of immunologic dysfunction. Clinical findings included the presence of arthritis, vasculitis, and erythema nodosum. Laboratory abnormalities included the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs tests, elevated latex fixations, and a circulating lupus type anticoagulant. Total hemolytic complement was markedly depressed in four patients. Analysis of complement (C) components C1-C9 and factor B demonstrated significant reduction of only C3 and factor B. By crossed-immunoelectrophoresis, both C3 and factor B, but not C4, were cleaved, indicating that C activation was occurring predominantly via the alternative pathway. The control proteins beta 1H and C3b inactivator were decreased in three of four patients with hypocomplementemia. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis.

Aged↗
PubMed1982

Bone and bone-marrow blood flow in chronic granulocytic leukemia and primary myelofibrosis.

Blood flow in hematopoietic bone marrow and in nonhematopoietic bone has been measured with a Xe-133 washout method in 20 patients with chronic granulocytic leukemia (CGL) and in seven with primary myelofibrosis. Age-matched healthy persons served as controls. Bone-marrow blood flow in CGL was dependent upon the phase of the disease. In the metamorphosis phase, bone-marrow blood flow was high compared with that in the well-controlled phase. Apart from the initial phase, the mean values for bone blood flow in CGL were increased compared with the values of the healthy controls. In myelofibrosis the bone blood flow was also increased. Bone-marrow blood flow in these diseases was dependent upon the cellularity of bone marrow as measured morphometrically.

Adult↗
PubMed1982

Bone-marrow imaging with indium-111 chloride in aplastic anemia and myelofibrosis: concise communication.

Twenty-nine patients with aplastic anemia and 11 patients with myelofibrosis were evaluated with indium-111 chloride bone-marrow imaging, ferrokinetics, and bone-marrow core biopsies. There was good correlation between the erythrocyte cellularity of the marrow and the In-111 bone-marrow scan grades in most patients. In some, the overall scan grade tended to underestimate the erythroid elements because the core biopsy had been taken from the area of the greatest radionuclide concentration on the scan. In patients with aplastic anemia, there was good correlation between the plasma iron clearance t1/2 and the scan grade. Less agreement was found in the comparison between the Fe-59 sacral and organ counts and the red-cell iron utilization. In patients with myelofibrosis, there was poor correlation between the surface counts over the sacrum and the red-cell iron utilization. Plasma iron clearances were abnormally short and were unrelated to the transferrin saturation levels. Eighteen patients were studied several times to evaluate their responses to steroid therapy. In all, there was good correlation between the bone-marrow imaging, the erythrocyte cellularity, ferrokinetics, and the patient's response to therapy. Indium-111 bone-marrow imaging is useful both in evaluating marrow erythroid activity and in following the response to therapy in patients with these diseases.

Adolescent↗
PubMed1982

Mechanism of extramedullary haematopoiesis in rabbits with saponin-induced myelofibrosis and myeloid metaplasia.

The saponin-induced myelofibrosis and myeloid metaplasia model in the rabbit was used to study mechanisms of extramedullary haematopoiesis. Haematopoietic progenitor cells, erythroid colony forming units (CFU-E) and burst forming units (BFU-E) were assayed serially in the peripheral blood, spleen and bone marrow after saponin administration, employing the in vitro methylcellulose culture technique. Animals that had undergone splenectomy prior to saponin administration were also studied. The results demonstrated increases of progenitor cells in the blood and spleen and a simultaneous depletion of such cells in marrow after saponin treatment. The results in splenectomized animals were similar to those observed in non-splenectomized animals after saponin administration. The findings indicate that following saponin administration there is a release of CFU-E and BFU-E from bone marrow into periphery and probably deposition in the spleen, and suggest that myeloid metaplasia in myelofibrosis may result from colonization of extramedullary sites originating from the bone marrow.

Animals↗
PubMed1982

The ultrastructure of radiation-induced endosteal myelofibrosis in the dog.

A rapidly developing, progressive form of endosteal myelofibrosis (MF) (with myeloid metaplasia) has been shown to occur at low frequency (approximately 4%) in dogs exposed continuously to low daily doses (10 R/day) of whole-body gamma irradiation. We report in this study the morphological details of the endosteal surface during both preclinical and clinical phases of developing MF by combination light microscopy and scanning/transmission electron microscopy. Pronounced alterations of the endosteum were observed and included: (1) during the early preclinical phases, a progressive time-dependent transition of the endosteal surface from predominantly resting to actively formative and resorptive states; and (2) during the late preclinical phase, aberrant autonomous osteogenic process(es) characterized by a marked reduction in the resorptive, osteoclast-associated endosteal areas occurring concomitantly with further increases in formative areas of the endosteum. Localized patches of overlapping, morphologically transformed endosteal cells (i.e., round-osteoblastic to branched-reticular shaped) were observed within the morphologically reactive, formative endosteum. Osteogenic-endosteal changes coincided with major restructuring of the hematopoietic parenchyma and supporting stromal network. We discuss the possibility that the early occurring endosteal changes are causally linked to normal reparative functions that operate during regenerative hematopoiesis following local and systemic injury. Based on morphological data collected during the late preclinical phase, we speculate that the mechanism of myelofibrosis induction involves the failure to terminate early osteogenic-dependent repair sequences.

Animals↗
PubMed1981

[Splenectomy in idiopathic thrombopenic purpura and in myelofibrosis. A retrospective study of platelet increase, hemorrhagic complications and mortality].

In a retrospective study of 39 splenectomies, patients with increased blood cell breakdown (13 cases of idiopathic thrombocytopenic purpura (ITP), 5 cases of hereditary spherocytosis, 2 of Felty's syndrome and 2 of autoimmune hemolytic anemia) were compared with those patients also presenting decreased blood cell production [14 cases of myelofibrosis (MF) with splenomegaly and 3 cases of advanced chronic myelogeneous leukemia (CML)]. Platelet regeneration post-operatively was significantly (p less than 0.01) more rapid in the ITP than in the MF group. Only 1/22 patients in the ITP group had major post-operative complications as compared to 10/17 in the MF group. None of the patients in the ITP group died within 25 days of operation as compared to 5/17 in the MF group. Only 3/22 patients in the ITP group lost more than 800 ml of blood during the operation as compared to 8/17 with MF. No statistically significant higher blood loss was found in patients with less than 30 x 10(9) platelets/l preoperatively, compared to those with higher platelet counts. However, correlation between the splenic weights and amount of blood loss was statistically significant (p less than 0.01). Thus, splenectomy seems much better tolerated in patients with ITP, even if platelets are low, than in patients with myelofibrosis.

Adult↗
PubMed1980

The radionindium bone-marrow image in acute (malignant) myelofibrosis.

A patient with a rapidly fatal case of acute myelofibrosis had a radioindium bone-marrow image that unexpectedly showed a normal pattern of distribution, unlike the more typical forms of chronic myelofibrosis or agnogenic myeloid metaplasia. The rapid progression of the disease and the unique handling of radioindium by the reticuloendothelial system may explain the discordant findings.

Acute Disease↗
PubMed1980

Urinary hydroxyproline excretion in myelofibrosis.

Urinary hydroxyproline measurements were performed in a group of health volunteers as well as patients with cancer and myelofibrosis. Patients in whom there was no metastatic involvement of bone marrow excreted an amount of hydroxyproline not different from that of the control group. Those who had marrow metastasis produced elevated levels of hydroxyproline; the highest excretions were observed when marrow fibrosis was associated with metastasis. These results contrasted with those observed in agnogenic myeloid metaplasia patients whose excretions were equivalent to the control group. The result suggests differences in the pathogenesis of myelofibrosis and a technique potentially useful for distinguishing between patients who may otherwise be diagnostic problems.

Bone Neoplasms↗
PubMed1995

[Hematological remission of primary myelofibrosis with antiphospholipid antibody following treatment of azathioprine].

A 46-years-old woman was admitted with purpura, nasal bleeding, gum bleeding, gross hematuria, cerebral hemorrhage, and cerebral infarction. The data of her peripheral blood were as follows: WBC 17,500/microliters, Hb 5.1 g/dl, PLT 0.3 x 10(4)/microliters, LDH 924 IU/l. Primary myelofibrosis was diagnosed because of bone marrow fibrosis and extramedullary hematopoiesis of the spleen. Furthermore, she revealed marked thrombocytopenia and no response to platelet transfusion, so association of idiopathic thrombocytopenic purpura (ITP) was considered. Though she was treated with high dose predonisolone, high dose gamma-globulin, and splenectomy, no hematological improvement was achieved. Administration of azathioprine (100 mg/day) was begun and 2 weeks later, her white blood cell count was approximately 10,000/microliters, the platelet count 2.0 x 10(4)/microliters, and no bleeding focus was found. Four weeks later, her hemoglobin content was 13.0 g/dl without blood transfusion. The diagnosis of antiphospholipid syndrome, rather than ITP, was made because of anticardiolipin-beta 2GPI complex antibody and cerebral infarction. It is interesting that immunosuppressant was effective both in primary myelofibrosis and in antiphospholipid syndrome.

Antibodies, Antiphospholipid↗
PubMed1995

[Idiopathic myelofibrosis with extramedullary hematopoiesis foci in the skin and testicles. Report of a case].

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.

Adult↗
PubMed1994

Prognostic significance of bone marrow reticulin fibres in idiopathic myelofibrosis: evaluation of clinicopathological parameters in a scoring system.

A clinicopathological scoring system was performed for obtaining a better estimate of prognosis in 50 patients with idiopathic myelofibrosis (IMF). Laboratory parameters including Hb-level, leukocyte and platelet counts, percentage of blast cells in peripheral blood, spleen and liver size, and a semiquantitative histological grading of reticulin fibre content of bone marrow biopsies taken at the time of initial diagnosis were analysed. Based upon these haematological and histological parameters three prognostic groups could be categorized with a significantly different survival (low-risk group with 21 patients = 75 months; medium-risk group with 18 patients = 51 months, and 11 patients in a high-risk group = 18 months). In an univariate (log rank test) and in a multivariate regression analysis the Hb-concentration, mild splenomegaly (less than 5 cm) and a higher grade of bone marrow reticulin content proved to be important prognostic parameters, whilst leukopenia, thrombocytopenia and the presence of peripheral blast cells were only of prognostic significance within the first 6 months from initial diagnosis. It was concluded that the increase of reticulin fibre deposition in bone marrow together with anaemia and mild splenomegaly could be responsible for a progressively worse life-expectancy of high-risk patients with idiopathic myelofibrosis.

Adult↗
PubMed1994

Risks and benefits of splenectomy in myelofibrosis: an analysis of 39 cases.

From 1980 to 1993, 39 splenectomies were performed in the Department of Visceral Surgery of Saint-Louis Hospital, in patients referred for myelofibrosis associated with myeloid splenomegaly. The short term morbidity was considerable: 33 serious haemorrhagic, infectious or thrombotic complications including 5 fatal accidents were observed in 18 patients. Severe thrombotic or infectious complications leading to 6 further deaths occurred in 8 patients over the two years following splenectomy, while six cases of acute leukaemia appeared between 6 months and 3 years after splenectomy. In 40% of cases with regular follow-up, the operation did not provide any haematological improvement and all these patients died. Only patients with minimally progressive or stable myelofibrosis and residual marrow activity in isotope studies showed an amelioration of general status with relief of pain and reduction of transfusional requirements. The indication for splenectomy should therefore probably be limited to such cases.

Adult↗
PubMed1994

[The beneficial effect of 1,25-dihydroxycholecalciferol on the excess of blasts and the myelofibrosis in 2 cases of chronic myeloproliferative syndrome].

The active metabolite of vitamin D3 1.25 dehydroxycholecalciferol (1.25DHCC) is a potent inducer of monocytic differentiation of the myeloid blasts "in vitro". Likewise the inhibiting role of vitamin D3 on bone marrow fibrosis by, among others, a stimulating effect of macrophagic activity is known. However, these actions have seldom been clinically demonstrated. Two cases of chronic myeloproliferative syndromes in which treatment with 1.25DHCC was effective are presented. In the first case, one patient with polycythemia vera with myelofibrosis and focal blastosis in the bone marrow achieved disappearance of the excess of blasts and a reduction in the fibrosis (grade III to grade I) upon treatment. In the second case, idiopathic myelofibrosis, also with focal blastosis in the bone marrow biopsy, there was no regression of the fibrosis but the blastosis did disappear. It was concluded that 1.25DHCC may constitute an interesting treatment in this group of diseases through the following two mechanisms: limitation of the fibrosis and delay of blast transformation.

Anemia, Refractory, with Excess of Blasts↗