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PubMed2004

Bone marrow angiogenesis and its clinical correlates in myelofibrosis with myeloid metaplasia.

BACKGROUND AND OBJECTIVES: Previous retrospective studies have indicated markedly increased bone marrow angiogenesis (BMA) in myelofibrosis with myeloid metaplasia (MMM). This issue is further examined in the current prospective study and clinico-pathological correlates sought. DESIGN AND METHODS: This was a prospective single institutional study of 66 patients with bone marrow biopsy-proven MMM who were consecutively accrued. Bone marrow angiogenesis was evaluated by assessing microvessel density through immunohistochemical staining for the CD34 antigen. Laboratory and clinical information was collected concurrently. RESULTS: The 66 patients (median age 62 years, 46 males) included 36 with fibrotic phase agnogenic myeloid metaplasia (AMM), 6 with cellular phase AMM, 4 with hypocellular variant AMM, 10 with post-polycythemic myeloid metaplasia, and 10 with post-thrombocythemic myeloid metaplasia. Overall, increased BMA was documented in 61 patients (92%). All of the aforementioned sub-categories of MMM were similarly affected in terms of either grade 3 or 4 BMA but differed in the prevalence of grade 4 BMA (25%, 0%, 0%, 10%, and 10%, respectively). In a univariate analysis the only histological feature that significantly correlated with BMA was the degree of megakaryocyte clustering. Among clinical features, increased BMA was significantly associated with younger age of the patient, lower hemoglobin level, intact spleen, and absence of active therapy. On multivariate analysis, only the latter two retained their significance. INTERPRETATION AND CONCLUSIONS: The current prospective study confirms the consistent association of BMA with MMM and suggests that the process starts early, is progressive, and might be dampened by both splenectomy and drug therapy. The study also suggests either a megakaryocyte origin or a megakaryocyte effect for the putative angiogenic cytokine.

Age Factors↗
PubMed2005

Aberrant expression of platelet-derived growth factor (PDGF) and PDGF receptor-alpha is associated with advanced bone marrow fibrosis in idiopathic myelofibrosis.

The expression of members of the platelet-derived growth factor (PDGF) system in bone marrow cells derived from Idiopathic myelofibrosis (IMF) has been investigated by real-time RT-PCR. Increased expression of PDGFs and the corresponding PDGF receptor a could be demonstrated to be a feature of advanced fibrosis in IMF that is not demonstrable in the prefibrotic phase of the disease.

Disease Progression↗
PubMedDate not supplied

Prevention of hepatic infarction as acute-phase complication of TIPS by temporary balloon occlusion in a patient with primary myelofibrosis.

Severe acute liver dysfunction occurred following transjugular intrahepatic portosystemic shunt (TIPS) creation in a patient with massive ascites due to portal hypertension associated with primary myelofibrosis. On US and TIPS venography, we considered that the acute liver ischemia was induced by TIPS. To avoid diffuse hepatic infarction and irreversible liver damage, a balloon catheter was inserted transjugularly into the TIPS tract and occluded it to increase portal venous flow toward the peripheral liver parenchyma. The laboratory data indicating hepatic dysfunction were improved after the procedure. We should pay attention to the possible occurrence of acute hepatic ischemia and infarction after TIPS creation even in a case of noncirrhotic portal hypertension. In such cases, temporary balloon occlusion of TIPS is an effective therapeutic method, probably as a result of inducing the development of arterial compensation through the peribiliary plexus.

Acute-Phase Reaction↗
PubMed2005

[Clinical features and prognostic factors in primary myelofibrosis patients under 45 years old].

OBJECTIVE: To investigate the clinical and hematological features and identify the prognostic factors associated with short-term survival in primary myelofibrosis (PMF) patients under 45 years old. METHODS: The clinical manifestations, laboratory parameters, and survival were retrospectively analyzed in 56 PMF patients under 45 years old. Univariate analysis of prognostic factors was performed using Logrank test and multivariate analysis using COX model. RESULTS: Of the 56 patients, 27 were males and 29 females. The range of age was from 20 to 45 years (median 38 years). 84% of the patients were anemic and 66% Hb < 100 g/L. 32% of the patients had constitutional symptoms including fever, night sweats and weight loss. The median survival was 69 months (95% CI 11-127). By univariate analysis, Hb < 100 g/L, platelet count < 100 x 10(9)/L, WBC < 10 x 10(9)/L, constitutional symptoms and the duration from first signs to diagnosis < or = 6 months were associated with poor prognosis. By multivariate analysis, only Hb < 100 g/L and constitutional symptoms were independently associated with short survival. With these two adverse prognostic factors, the patients could be separated into a high risk and a low risk groups, and the median survivals were 16 and 88 months, respectively (P < 0.001). Using these two factors to predict the less than 3-years survival for individual patient, the sensitivity, specificity and positive predictive value were 67%, 100% and 100%, respectively. CONCLUSION: Hb < 100 g/L and constitutional symptoms in PMF patients under 45 years old were significantly associated with short survival and poor prognosis. These two prognostic factors enabled to separate patients into a high and a low risk groups. The survival of high-risk patients was less than three years.

Adult↗
PubMed1992

[Successful low-dose etoposide therapy for a case of myelofibrosis with chronic myelogenous leukemia megakaryocytic predominance type].

A 38-year-old woman presented with ear pain and vertigo. No hepatosplenomegaly or lymphadenopathy were found, but her platelet counts markedly rose to 414 x 10(4)/microliters with an increase of megakaryocytes in the bone marrow (859/microliters). Cytogenetic assay revealed positive Ph1 chromosome and rearrangement of the break point cluster region (bcr). Although platelet counts remained under 100 x 10(4)/microliters after the administration of carboquone, a high fever and pancytopenia appeared 31 months later. Bone marrow biopsy showed marked myelofibrosis which was improved by low dose etoposide. This case was thought to be Ph1 positive ET, but it was more compatible with CML megakaryocytic predominance type according to the newly proposed "Hannover criteria for myeloproliferative disorders" and cytogenetic assay.

Adult↗
PubMed1992

[Essential thrombocythemia that transformed to myelofibrosis after three years].

A 66-year-old man was presented with thrombocytosis in February, 1988. Laboratory examinations on admission revealed a white blood cell count of 17,700/microliters and a platelet count of 274.4 x 10(4)/microliters. Bone marrow aspirates showed an increase of megakaryocytes (1,294/microliters). There was no fibrosis or Ph1 chromosome. He was diagnosed as having essential thrombocythemia and was treated with thrombopheresis, carboquone and ranimustine (MCNU). Subsequently his platelet count was well controlled approximately for three years. He was readmitted because of pyrexia and left hypochondralgia in February 1991. Physical examination revealed hepatosplenomegaly. Peripheral blood revealed leukoerythroblastosis associated with the occurrence of tear drop cells. Bone marrow aspiration resulted in a dry tap and the biopsy specimen showed reticulin fibrosis. This is a fairly rare case of essential thrombocythemia that transformed to myelofibrosis.

Aged↗
PubMed1992

Abdominal ultrasonography in myelofibrosis.

The findings at abdominal ultrasonography (US) in 40 patients with myelofibrosis were reviewed, 20 patients being examined at initial diagnosis and 31 at later stages. Splenomegaly was found in 80% at initial diagnosis and in 97% at later stages. The spleen of 2 patients appeared homogeneously hypoechoic and inhomogeneous in one. Focal splenic lesions were seen in 5, and calcifications in 6. Mixed splenic lesions proved to be metastases in one and hyperechoic lesions in another patient were due to extramedullary hematopoiesis. Hepatomegaly was found in 25% at primary diagnosis and in 39% at later stages. Focal hepatic lesions were seen in 7 patients, and proved to be metastases in 3. The focal lesions in 2 of these patients were extramedullary hematopoiesis, which was hypoechoic in one and hyperechoic in the other. Ascites was seen in 4 patients and lymphadenopathy in one. US could not reliably differentiate between extramedullary hematopoiesis and malignancy. Fine-needle biopsy may be performed for definitive diagnosis.

Abdomen↗
PubMed1992

[Improvement of bone marrow hematopoiesis in idiopathic myelofibrosis with prednisolone].

A 64 year-old female patient of idiopathic myelofibrosis (IMF), who had had rapidly progressing massive splenomegaly and severe pancytopenia refractory to blood transfusion, was treated with PSL 0.6 mg/kg/day for a month, being significantly improved not only symptomatically and hematologically but also in bone marrow hematopoiesis. Although the effectiveness of PSL to restoration of bone marrow hematopoiesis has been almost unknown, long term oral PSL should be tried for a certain phase of IMF.

Bone Marrow↗
PubMed2005

[Intracranial extramedullary hematopoiesis following bilateral chronic subdural hematoma in a patient with idiopathic myelofibrosis].

This is a case of remarkable intracranial extramedullary hematopoiesis (EMH) in a 63-year-old male patient with a history of idiopathic myelofibrosis. Intracranial EMH was suspected following cranial computed tomography and magnetic resonance imaging and was found in the same sites as the bilateral subdural hematomas. The patient subsequently received whole brain irradiation (30 Gy). The lesion was controlled for one and a half years. After the patient died from bacterial infection, intracranial EMH was confirmed in autopsy and histological diagnoses.

Hematoma, Subdural, Chronic↗
PubMed2006

[A case of dermatomyositis associated with chronic idiopathic myelofibrosis].

A 74-year old woman had been suffering from chronic idiopathic myelofibrosis (CIMF) for three years before noticing skin rash and subsequent muscle weakness. On admission, purplish and erythematous skin rash was seen spreading over large parts of her body, including the face, chest, back and extremities. She could not stand up without assistance due to weakness. On laboratory examination, her hemoglobin was found to be 11.8g/dl, platelet 17,000/microl, WBC 22,500/microl (with blast cells), and CK 1,757 IU/I. Auto-antibodies including antinuclear antibody, Jo-I antibody and rheumatoid factors were negative. Abdominal CT revealed giant splenomegaly. She was diagnosed as having dermatomyositis (DM) associated with CIMF. Although administration of prednisolone followed by methyl-prednisolone pulse therapy ameliorated the weakness and skin rash, WBC increased to 35,000/ microl. In case of worsening of CIMF, azathioprine (AZP) administered. This decreased the WBC count to 13,700/microl and the CK to 49 IU/I 40 days after the administration. Patients with CIMF have an increased incidence of complications of other autoimmune diseases, indicating that the immunological mechanism plays some roles in the progression of the disease. There has been only one other reported case of DM complication. This case suggests usefulness of AZP treatment combined with prednisolone in these patients.

Aged↗
PubMed2006

Idiopathic myelofibrosis without dacryocytes.

Idiopathic myelofibrosis (IMF) typically presents with marrow fibrosis, splenomegaly, progressive anemia, and a leukoerythroblastic blood smear with dacryocytes. We present a patient with IMF who did not have dacryocytes.

Aged↗
PubMed2006

[Low dose whole lung irradiation for intractable pleural effusion due to idiopathic myelofibrosis].

A 63-year-old man with idiopathic myelofibrosis, diagnosed on Sept. 2004 and treated with hydroxyurea (HU), was admitted to our hospital on March 2005 because of dyspnea and pleural effusion on the right side. Effusion analysis revealed hemothorax due to extramedullary hematopoiesis or bleeding. We performed a thoracoscopic pleural lung biopsy and pleurodesis with minocycline after surgical evacuation. However, bilateral pleural effusion appeared only 2 weeks later, which was thought to be extramedullary hematopoiesis or acute transformation. We adopted a treatment strategy with low dose whole lung irradiation (4.25 Gy/5 fractions) which was followed by complete disappearance of the bilateral pleural effusion. Our present case suggests that low dose whole lung irradiation may be an effective and safe therapy for intractable pleural effusion due to extramedullary hematopoiesis.

Hematopoiesis, Extramedullary↗
PubMed2006

Thalidomide versus placebo in myeloid metaplasia with myelofibrosis: a prospective, randomized, double-blind, multicenter study.

BACKGROUND AND OBJECTIVES: In non-randomized studies, thalidomide appeared to be effective in myeloid metaplasia with myelofibrosis (MMM). We compared thalidomide to placebo for treatment of anemia in MMM. DESIGN AND METHODS: A prospective phase II B, randomized double-blind multicenter trial comparing thalidomide 400 mg/d with placebo for 180 days was conducted in 52 anemic patients (hemoglobin pounds Sterling 9 g/dL or transfused). The main outcome measure was a 2 g/L increase in hemoglobin or 20% reduction in transfusions. RESULTS: In the thalidomide group only 10 patients completed 6 months of treatment. At 180 days, in an intention-to-treat analysis, no difference was observed between the thalidomide and placebo groups as regards improvement of hemoglobin levels (one patient in each group) or reduction of red blood cell transfusions (three vs five patients, respectively). The spleen size, determined by ultrasonography, increased significantly less in the thalidomide group than in the placebo group (p < 0.05). Thalidomide had no apparent benefit on the Dupriez score, the severity score, survival, death, or any other clinical or biological parameter. Somnolence, gastro-intestinal signs, weight gain, and edema were significantly more frequent in the thalidomide group. Outpatient discontinuation of thalidomide was significantly correlated with a high severity score > 4 (odds ratio, OR = 16; p < 0.01), and g-glutamyl transferase levels > 40 IU/L (OR = 12; p < 0.05). INTERPRETATION AND CONCLUSIONS: Thalidomide (200-400 mg/d) does not demonstrate substantial efficacy in anemic MMM patients. The natural history of disease in the placebo group revealed spontaneous periods of remission of anemia. Tolerance of thalidomide was significantly correlated wih the severity and liver involvement of the disease.

Angiogenesis Inhibitors↗
PubMed1990

Idiopathic myelofibrosis serum induces increased DNA synthesis in BALB/c 3T3 cells.

The effect of heated serum at a concentration of 10% in culture on the in vitro growth of confluent Balb/c 3T3 cells was studied in nine patients with Idiopathic Myelofibrosis and ten normal subjects. Patients showed significant increase in the mitogenic activity in comparison with normals. The growth factors conceivably implied for the observed effect are discussed. Particular attention is paid to Platelet-Derived Growth Factor from which serum mitogenic activity is primarily derived and is thought to take part in the genesis of bone marrow fibrosis.

Aged↗
PubMed1991

[Primary myelofibrosis transforming into multiple subcutaneous monoblastoma--a case report].

A 83-year-old man was diagnosed with primary myelofibrosis based on the presence of leukoerythroblastosis, splenomegaly, chromosome 46 XY, a dry tap bone marrow aspiration and fibrosis on bone marrow biopsy, when he was admitted for herpes zoster in June 1987. He was admitted for a second time with multiple subcutaneous tumors over his entire body in July, 1989. He had mild splenomegaly, but no hepatomegaly nor lymphadenopathy. Laboratory tests were as follows: RBC 214 x 10(4)/microliters, Hb 5.1 g/dl, Ht 17.7%, WBC 3,200/microliters with leukoerythroblastosis, platelets 11.6 x 10(4)/microliters, s-lysozyme 251 micrograms/ml, u-lysozyme 770 micrograms/ml, NAP ratio 98%, score 278. Bone marrow aspiration resulted in a dry tap. Bone marrow biopsy showed marked fibrosis. Histologic examination of subcutaneous tumor biopsy specimens revealed a diffuse infiltration of monocytes with flexuous nuclei. These cells were positive for alpha-naphtyl butyrate esterase stain, and negative for peroxidase, alpha-naphtol ASD chloroacetate esterase stain and platelet glycoprotein IIb/IIIa stain (APAAP). Ultrastructurally, these cells were mostly monocytes and promonocytes, while phenotypically, CD11b, CD13, CD14, CD33 and HLA-DR were positive. These date indicated that the subcutaneous tumors originated from monocytes.

Aged↗
PubMed1991

[Essential thrombocythemia transformed to myelofibrosis with myeloid metaplasia after seven years].

A 29-year-old male was diagnosed as having essential thrombocythemia (ET) in 1975. From that time, his platelet count gradually increased to more than 2 X 10(6)/microliter until 1979. However, his platelet count gradually decreased to less than 6 X 10(5)/microliter in 1985. Also, in 1982, erythroblasts and immature myeloid cells began to appear in the peripheral blood, and the liver and spleen became palpable in 1985. Bone marrow then revealed osteomyelosclerosis. These findings suggested that ET had transformed to myelofibrosis with myeloid metaplasia. Increased hepatosplenomegaly was accompanied by the appearance of ascites in June, 1988, and an esophageal varix ruptured in December of the same year. The varix was resected and the spleen was removed. After the operation, ascites did not recur and his condition became stable. Portal hypertension in this patient was considered to be due mainly to increased blood flow from the enlarged spleen.

Humans↗