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A case of pyoderma gangrenosum associated with idiopathic myelofibrosis is described. It is important to recognise it, since the treatment with corticosteroids and sulphonamides is frequently effective.
BACKGROUND: In order to determine whether recombinant human erythropoietin (rHuEPO) may play a role in treating anemia in idiopathic myelofibrosis (IMF), a pilot study using high doses of rHuEPO was conducted on patients with IMF. METHODS AND RESULTS: From September, 1990 to December, 1992, 7 patients (6 males and 1 female, median age 68 years) affected by IMF entered the trial. RHuEPO was administered subcutaneously 5 days a week at a dosage of 160 U/kg daily for three months. Out of 7 patients, 4 obtained a response. These responders received additional maintenance treatment with rHuEPO until relapse. Response duration was 6, 7, 10+, and 16 months, respectively. Treatment was well-tolerated. CONCLUSIONS: Our data suggest that rHuEPO may play a role in the treatment of anemia in some IMF patients.
Primary myelofibrosis (PMF) is regarded as a chronic myeloproliferative disorder. It is characterized by marrow fibrosis, leukoerythroblastosis, tear drop erythrocytes and extramedullary hematopoiesis. Most patients are in their late 50s when first diagnosed. Pediatric PMF is said to be quite rare. Here describe a female infant with PMF. The patient was born on Aug. 7, 1991. The pregnancy and delivery were uneventful. Hepatomegaly was noted soon after birth. Combined blood counts showed polycythemia and leukocytosis. It was thought to be extramedullary hematopoiesis due to intrauterine infection. She was followed up in another hospital, but since her condition was unchanged she was admitted to our hospital for further medical examinations at age 7 months. On the peripheral blood smear, there were tear drop erythrocytes, normoblasts and early myeloid elements. Repeated bone marrow aspirations were dry taps. This case presented the classical findings of fibrosis of the bone marrow on bone marrow biopsy. She is in good health without any therapy until now. A review of 7 cases of PMF, including our case, in Japanese children was made and discussed in comparison to adult cases.
PURPOSE: To analyse the status of iron deposits at diagnosis in patients with idiopathic myelofibrosis (IM). PATIENTS AND METHODS: The study included twenty consecutive patients with IM. Haemoglobin, MCV, serum iron and serum ferritin were determined in each case. Perls stain was used to evaluate iron deposits in bone marrow samples attained by aspiration or biopsy. An attempt was made to correlate the semiquantitative estimation of bone marrow iron with the peripheral blood values connected with iron deposits. RESULTS: Anaemia was present in 15 patients, but in most cases MCV and serum iron were normal. Iron deposits in bone marrow aspirate could be evaluated only in one patient as in the remainders no material was obtained. The semiquantitative estimation of iron in bone marrow biopsy samples showed diminished or absent deposits in 15 cases (75%). In contrast, none of the patients had decreased serum ferritin levels. No correlation was found between bone marrow iron and the peripheral blood values related to iron deposits. CONCLUSION: Most IM patients have decreased bone marrow iron deposits at diagnosis. However, as in chronic myelogenous leukaemia and in essential thrombocytopenia, such finding does not mean a true iron deficiency status.
Thrombocytopenia and splenomegaly improved in one of the four patients receiving 1 alpha-hydroxyvitamin D3 (1 alpha(OH)D3) for treatment of primary myelofibrosis (PMF). We compared the clinical results with the in vitro effects of vitamin D3 metabolites on the growth and collagen synthesis of bone marrow fibroblasts. The effects of vitamin D3 metabolites on control human bone marrow fibroblasts were first studied in vitro. On the growth, 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) and 24,25-dihydroxyvitamin D3 (24,25(OH)2D3) inhibited the platelet derived growth factor (PDGF) or human serum-induced proliferation at the concentrations of more than 10(-10) M, while in the presence of transforming growth factor-beta (TGF-beta) the inhibitory effects were mild or non-inhibitory. Both vitamin D3 metabolites inhibited procollagen synthesis at a concentration of more than 10(-8) M. The effect of 1,25(OH)2D3 on the PMF patients was examined. In two of the four patients, the human serum-induced growth inhibitory effect was observed at the concentration of 10(-8) M, in one patient no inhibition was observed and in one patient inhibition was observed at 10(-10) M, as was observed in the control donors. During the treatment with 1 alpha(OH)D3 the serum level of 1,25(OH)2D3 was 1-2 x 10(-10) M. These findings suggest that a minority of PMF patients have marrow fibroblasts with growth sensitivity to a pharmacological level of vitamin D3 and could be treated with 1 alpha(OH)D3 with some clinical improvements.
A 53-year-old male was admitted to our hospital with abdominal pain. Physical examination revealed marked splenomegaly. The white blood cell count increased to 5.8 x 10(4)/microliters. Bone marrow biopsy showed hypercellularity with a moderate increase in reticulin fiber. Chromosomal analysis showed 47, XY, +9q-, -9q- without Ph1 chromosome and bcr-abl rearrangement. MCNU therapy was successful in reducing the white blood cell count and splenomegaly. It is likely that the diagnosis of our patient is compatible with the neutrophilic myelofibrosis described by Stewart, et al.
Since according to the early studies, the outcome after splenectomy in the individual patient with myelofibrosis with myeloid metaplasia (MMM) is unpredictable, we assessed retrospectively the pre-intervention characteristics that best predicted adverse events, hematological consequences, and survival in 71 splenectomized MMM patients. The findings indicate that the operative risk of splenectomy for both mortality (8.4%) and morbidity (39.3%) was unpredictable. New hemorrhagic or thrombotic complications occurred in 16.9% of surviving patients and were predicted by age < 50 years, a normal to high platelet count (> 200 x 10(9)/l) and huge splenomegaly (> 16 cm from the costal margin). Massive liver enlargement occurred in 24% of patients and has to be expected in patients splenectomized for transfusion-dependent anemia. Anemia improved substantially in 45% and 52% of patients at 3 months and at 1 year, respectively, and was predicted by severe anemia, low platelet count (< 100 x 10(9)/l) or normal to high white blood cell (WBC) count (> 4 x 10(9)/l). Survival from splenectomy was superior in patients < 45 years with WBC < 10 x 10(9)/l count. An unexpectedly high rate of blastic transformation was observed. It accounted for 42.8% of the deaths. The results suggest trials for prophylactic cytoreductive treatment in young patients and when platelet count is normal to increased. Further study is needed for elucidating the possible role played by splenectomy in inducing blastic transformation.
A 62 year old male patient was previously admitted to another hospital in February 1989 because of palpitation. Peripheral blood examination revealed pancytopenia. Although the number of nucleated cells in the bone marrow aspirate was in the normal range. It contained 2.2% of blastic cells and dysplastic cells. He was diagnosed to be myelodysplastic syndrome with refractory anemia. He subsequently received repeated blood transfusions and other symptomatic treatments as an outpatient until 1990. When he was admitted to our hospital because of severe pancytopenia. The numbers of WBC, RBC, and platelets were 2,000/microliters, 134 x 10(4)/microliters, 2.9 x 10(4)/microliters respectively. Bone marrow aspiration resulted in dry tap, and biopsy at the iliac bone showed remarkable fibrosis with marked decrease of normal hematopoietic cells. Chromosome analysis revealed multiple aberrations such as 47XY, +8, 13q-, 14p+, 48XY, +8, +9, 13q+. The patient was treated with BHAC-AMP combination chemotherapy. After 3 cycles of the therapy, pancytopenia was improved and chromosomal aberration disappeared. This case was considered to be an acute myelofibrosis developed from myelodysplastic syndrome and worth to reporting with a review of literature, because drastic combination chemotherapy was extremely effective.
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.
Here we report a 65-year-old male patient with idiopathic myelofibrosis (IMF) accompanied with acromegaly. He was admitted because of anemia and splenomegaly. No favorable effects were observed when hydroyurea was administered. However, his symptoms were successfully treated by administration of vincristine and prednisolone (VP therapy), which is usually applied for choronic myelogenous leukemia in the accelerated phase. Therefore, VP therapy might be one possible for IMF. Since there have been several reports suggesting the possible association of hematological malignancies with acromegaly, we speculate that acromegaly was implicated in the initiation or progression of IMF in this case.
A 45-year-old man was admitted with high fever and leukocytosis in August 1993. The diagnosis of acute myelogenous leukemia (AML; M2) was made on the basis of morphological, cytochemical and immunological characteristics of the blasts in the bone marrow. The induction therapy with BHAC, daunorubicin, 6-MP was unsuccessful in achieving remission; the bone marrow biopsy specimen revealed the proliferation of the remaining leukemic cells and massive fibrosis accompanied with unusual megakaryocyte-like giant bizarre cells. These megakaryocyte-like giant cells were positive for myeloperoxidase and CD34, but not GPIIIa and factor VIII, indicating that those were derived from myelogenous stem cells. Following the low-dose Ara-C therapy, improvement of fibrosis and disappearance of these giant cells were observed in the bone marrow. After the reinduction therapy with high-dose Ara-C and MIT against markedly increased blasts, the patient died of systemic fungal infection. The presence of myelofibrosis and giant atypical blasts might allow resistance to therapy and poor prognosis.
We describe a case of early myeloid blastic transformation in a 64-year-old man suffering from myelofibrosis with myeloid metaplasia. Both chronic and blastic phase cytogenetic analysis showed trisomy 13 to be the sole chromosome aberration. A potential role for this rare abnormality in determining such an unusually poor clinical outcome is discussed.
The authors present the case of a 73 year old man with primary myelofibrosis, pulmonary fibrosis, and acquired sea-blue histiocytosis, who died of respiratory failure. Pathology of the lungs revealed infiltration by sea-blue histiocytes, and fibrosis in the alveolar septa, and clumps of these cells filling the alveolar spaces. Megakaryocytes were also occasionally observed in the alveolar capillaries.
When gallium is injected into a patient who has received many transfusions, the resultant scan appears similar to a bone scan with renal excretion. This case report presents a 51-year-old man with myelofibrosis and myeloid metaplasia. Gullium-67 and 52Fe scans with 99mTc-pyrophosphate bone scintigrams are included.
The published experience of allogeneic bone marrow transplantation for primary myelofibrosis (PMF) is limited. Three patients (24-49 years) with PMF received allogeneic marrow grafts from HLA-identical sibling donors after conditioning with 110 mg/m2 melphalan and 1050 cGy total-body irradiation (TBI). Donor marrow was not depleted of T cells, and graft-versus-host disease (GVHD) prophylaxis comprised cyclosporine and methotrexate. None of the patients was splenectomized prior to the transplant. Two patients received G-CSF post-transplant to hasten neutrophil recovery. One patient died of multi-organ failure 23 days post-transplant. Hematopoietic recovery was relatively slow in the other two who had gradual resolution of the marrow fibrosis over several months. One of the two died of overwhelming pneumococcal sepsis within 2 weeks of stopping prophylactic penicillin 31 months post-transplant. The other patient is alive and well 20 months post-transplant with a Karnofsky score of 100% and no fibrosis of the marrow. We conclude that PMF is correctable by allogeneic BMT. Hematologic recovery post-transplant is slow, but counts may normalize with time without the need for splenectomy.
Here we present the case of a patient affected with a unique association of Sweet's syndrome, idiopathic myelofibrosis, spindle-cell thymoma, myasthenia gravis and Good's syndrome (a rare form of thymoma-related, combined immunodeficiency presenting with recurrent respiratory infections). Conventional therapies (corticosteroids, colchicine, DDS, clofazimine) were ineffective or were contraindicated. Treatment with etretinate (50 mg/day) proved effective on skin lesions. Moreover, the patient's general condition unexpectedly improved, with long-lasting (11 months) suppression of respiratory infections and a slight but consistent improvement of hematological parameters such as Hct, MCV, Hb level, RBC, WBC and lymphocyte absolute counts.
Myelofibrosis with myeloid metaplasia (MMM) is a myeloproliferative disorder characterized by clonal expansion of hematopoiesis and marrow fibrosis. Previous results from our group have shown an increased production of two potent fibrogenic factors also involved in the regulation of primitive hematopoietic cells, namely transforming growth factor-beta1 (TGF-beta1) and basic fibroblast growth factor (bFGF), in patients with MMM. It is likely to assume that the myeloproliferation characteristic of this disease may result from an abnormal proliferation of CD34+ hematopoietic progenitors. Thus, we were particularly concerned in studying the gene and protein expression of these cytokines and their receptors in CD34+ progenitors purified from the peripheral blood of MMM patients by using semiquantitative reverse transcriptase-polymerase chain reaction and immunolabeling methods. Our data showed that the expression of TGF-beta1 is not altered in patients CD34+ cells; in contrast, the expression of TGF-beta type II receptor is significantly decreased in such cells, as compared with CD34+ cells from healthy subjects. Regarding bFGF, the very low expression of the cytokine and its type I and II receptors detected in normal CD34+ cells contrasts with that observed in patients' CD34+ cells, which is significantly higher. Our results might be a clue for a better understanding of the mechanism(s) involved in the dysregulation of hematopoiesis in MMM. Actually, the increased expression of bFGF and its receptors associated with the reduction of the TGF-beta binding receptor in CD34+ progenitors from MMM patients might facilitate the expansion of hematopoietic progenitors, not only by stimulating their growth and/or survival, but also by overcoming negative regulatory signals.
A 63-year-old male was admitted because of pneumonia. Peripheral blood findings showed pancytopenia with increase of blasts. A bone marrow specimen showed hypocellular marrow with increase of blasts. The blasts were positive for CD7, CD34, and HLA-DR and negative for other lymphoid antigens and myeloid antigens involving myeloperoxidase. Rearrangement of immunoglobulin heavy chain was demonstrated by Southern blotting analysis. T cell receptor beta, T cell receptor gamma and immunoglobulin light chain rearrangement were negative. A diagnosis of stem cell leukemia was made. In vitro, the blasts did not respond to recombinant human granulocyte colony-stimulating factor (rhG-CSF), cytarabine (Ara-C) and all-trans retinoic acid (ATRA). However, in the blasts of culture without cytokeins, CD33 expression was newly induced. Remission was not obtained by chemotherapies with cyclophosphamide, etoposide, prednisolone and Ara-C. Four months later, marrow specimens showed hypoplasty with myelofibrosis. One year later, the blasts showed CD33 expression with negative myeloperoxidase. The leukemia was transformed to minimally differentiated myeloid leukemia from stem cell leukemia. This condition was thought to be "smoldering leukemia" because of the slow development and refractoriness to chemotherapy. Nineteen months later the patient died due to respiratory failure by pneumonia and pulmonary bleeding despite therapy.