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PubMed1993

[Eosinophilic fasciitis (Shulman syndrome) as a rare differential diagnosis of sclerodermiform skin changes].

For 3 months, a 70-year-old woman had been affected by painless, symmetrical swellings in the limbs, spreading proximally, causing hardening of the skin and impaired joint movements, but no fever. Her general condition and findings on physical examination were according to age. ESR was 22/42 mm and there was a thrombocytosis (533,000/microliters) and eosinophilia (16%, absolute 2,140/microliters). Radiological and ultrasound examinations were normal. Skin biopsy revealed marked oedema of the connective tissue septa and perivascular lymphohistiocytic infiltrations, which--together with the other findings--suggested eosinophilic fasciitis. She was treated with prednisone, 100 mg daily, reduced gradually to 8 mg daily, as well as cimetidine, 800 mg daily, as immunomodulator, and intensive physiotherapy. The swellings and eosinophilia regressed within 8 weeks, but the hardening and impaired mobility persisted. Maintenance glucocorticoids are being continued.

Aged↗
PubMed1990

[Eosinophilic fasciitis (Shulman's syndrome)].

A 36-year-old man had noted a firm and painful swelling of the upper and lower limb, increasing over the previous few weeks. He had no fever, but the erythrocyte sedimentation rate was slightly increased (20 mm in the first hour). In addition there was a mild normochromic, normocytic anaemia (13 g/dl) and thrombocytosis (517,000/microliters). Gamma-globulin fraction was raised to 26%. The blood eosinophilia of 44% and the histological findings in a wedge biopsy of the lower leg (oedematous widened connective tissue septa, inflammatory infiltrate with eosinophilic granulocytes) established the diagnosis of eosinophilic fasciitis (Shulman syndrome). During oral treatment with prednisone (100 mg/d) the clinical symptoms regressed and the eosinophilia in peripheral blood disappeared. In the course of the subsequent seven months the prednisone dose was reduced gradually to 10 mg daily. But, because the symptoms did not entirely disappear the prednisone dose had to be increased temporarily for four times until there was a further, stepwise, reduction of the symptoms. But mild induration of the lower leg persisted. Maintenance treatment with prednisone, 10 mg daily, has been continued in order to avoid recurrence.

Adult↗
PubMed1998

Impaired expression of the thrombopoietin receptor by platelets from patients with polycythemia vera.

BACKGROUND: The cause of polycythemia vera, which originates from a multipotent hematopoietic progenitor cell, is unknown. Thrombopoietin is a hematopoietic growth factor that regulates the production of multipotent hematopoietic progenitor cells and platelets. To evaluate the possibility that an abnormality in thrombopoietin-mediated signal transduction might be involved in the pathogenesis of polycythemia vera, we examined thrombopoietin-induced tyrosine phosphorylation of proteins and the expression of the thrombopoietin receptor in platelets from patients with the disease. METHODS: Platelets were isolated from the blood of patients with polycythemia vera or other chronic myeloproliferative disorders and control subjects. The platelets were exposed to either thrombopoietin or thrombin and then lysed for analysis of tyrosine phosphorylation of platelet proteins and the expression of the proteins by means of immunoblotting. Expression of the thrombopoietin receptor (Mpl) by platelets and megakaryocytes was also assessed. RESULTS: Thrombopoietin-mediated tyrosine phosphorylation of proteins was impaired in platelets from 20 patients with polycythemia vera and 3 with idiopathic myelofibrosis, but not in 4 patients with essential thrombocytosis, 3 with chronic myelogenous leukemia, 6 with secondary erythrocytosis, 2 with iron-deficiency anemia, 4 with hemochromatosis, or 5 normal subjects. Thrombin-mediated tyrosine phosphorylation of proteins was intact in platelets from patients with polycythemia vera, and the tyrosine kinases and substrates involved in the process were present in normal amounts. However, expression of the platelet thrombopoietin receptor MpI was markedly reduced or absent in 34 of 34 patients with polycythemia vera and in 13 of 14 patients with idiopathic myelofibrosis. Impaired thrombopoietin-induced tyrosine phosphorylation of proteins in patients with these two diseases was uniformly associated with markedly reduced expression of MpI or the lack of its expression. In patients with polycythemia vera, reduced expression of MpI by platelets was associated with reduced expression of MpI by megakaryocytes. CONCLUSIONS: Reduced expression of the thrombopoietin receptor MpI is characteristic of polycythemia vera and idiopathic myelofibrosis. The abnormality appears to distinguish polycythemia vera from other-forms of erythrocytosis.

Adult↗
PubMed2003

Erythromelalgia precipitated by acral erythema in the setting of thrombocytopenia.

Erythromelalgia is a rare syndrome that is characterized by episodic attacks of burning pain in the distal extremities, which last from minutes to days and are precipitated by exercise, warmth, or limb dependency. There is a primary or idiopathic form and a secondary form that occur with myeloproliferative or other diseases. All previous reports about erythromelalgia that is the result of a myeloproliferative process have documented associated thrombocytosis. We describe a 40-year-old woman with myelodysplastic syndrome who experienced erythromelalgia in the setting of acral erythema and thrombocytopenia, first induced by chemotherapy and recurring after a radiation- and chemotherapy-based myeloablative regimen that was administered before a T-cell-depleted allogeneic bone marrow transplantation.

Adult↗
PubMed2000

Rab geranylgeranyl transferase alpha mutation in the gunmetal mouse reduces Rab prenylation and platelet synthesis.

Few molecular events important to platelet biogenesis have been identified. Mice homozygous for the spontaneous, recessive mutation gunmetal (gm) have prolonged bleeding, thrombocytopenia, and reduced platelet alpha- and delta-granule contents. Here we show by positional cloning that gm results from a G-->A substitution mutation in a splice acceptor site within the alpha-subunit of Rab geranylgeranyl transferase (Rabggta), an enzyme that attaches geranylgeranyl groups to Rab proteins. Most Rabggta mRNAs from gm tissues skipped exon 1 and lacked a start codon. Rabggta protein and Rab geranylgeranyl transferase (GGTase) activity were reduced 4-fold in gm platelets. Geranylgeranylation and membrane association of Rab27, a Rab GGTase substrate, were significantly decreased in gm platelets. These findings indicate that geranylgeranylation of Rab GTPases is critical for hemostasis. Rab GGTase inhibition may represent a new treatment for thrombocytosis and clotting disorders.

Alkyl and Aryl Transferases↗
PubMed2006

Genetic heterogeneity of granulocytes for the JAK2 V617F mutation in essential thrombocythaemia: implications for mutation detection in peripheral blood.

AIMS: The molecular pathogenesis of essential thrombocythaemia (ET) is heterogeneous. We aimed to determine the relative sensitivity of four separate molecular assays used to detect the presence of the JAK2 V617F mutation in peripheral blood from patients with essential thrombocythaemia and related myeloproliferative disorders. METHODS: Purified granulocytes from 60 patients were analysed for the presence of the JAK2 V617F mutation by direct sequencing, denaturing high-performance liquid chromatography (DHPLC), allele-specific polymerase chain reaction (PCR) and allele-specific enrichment. Clinical data were collected for all patients and correlated with assay results. RESULTS: Direct sequencing and DHPLC were relatively insensitive assays for mutation detection, together identifying only 53% of the JAK2 V617F positive cases of ET. Allele-specific PCR and allele-specific enrichment were significantly more sensitive assays and were able to identify additional ET patients that were positive for the JAK2 V617F mutation in only a minority of circulating granulocytes. Enrichment for the mutation was demonstrated in blood platelets from two of these patients. CONCLUSIONS: The observed biological difference in circulating granulocyte involvement by the JAK2 V617F clone necessitates a sensitive molecular assay for the diagnostic investigation of thrombocytosis.

Adult↗
PubMed1978

Electrolyte studies. 2. Potassium, chloride, and acid-base.

Hypokalemia plus elevated carbon dioxide (CO2) content usually indicates renal or gastrointestinal potassium (K+) loss. Hypokalemia plus decreased CO2 content usually means intestinal K+ loss. Hyperkalemia is common in metabolic acidosis and oliguric renal failure. With hemolysis or thrombocytosis, serum K+ concentration may be elevated while plasma concentration is normal. A CO2 value less than 18 mmole/liter suggests metabolic acidosis; a value greater than 30 mmole/liter suggests metabolic alkalosis.

Acid-Base Imbalance↗
PubMed1979

Antiplatelet drugs in thromboembolism.

Evidence is mounting that three drugs that inhibit platelet function--aspirin, dipyridamole, and sulfinpyrazine--have an antithrombotic effect in humans. Particularly in men, aspirin is beneficial in controlling transient ischemic attacks and stroke, and there is evidence that it may be effective in preventing thrombotic and embolic complication of hip surgery. It abolishes symptoms in peripheral ischemia associated with thrombocytosis and spontaneous platelet aggregation and may prove effective in coronary artery disease. When combined with oral anticoagulants, aspirin is more effective than oral anticoagulants alone in preventing systemic embolism in patients with prosthetic heart valves. Dipyridamole in combination with oral anticoagulants reduces the incidence of systemic embolism after prosthetic heart valve replacement. Sulfinpyrazone reduces the incidence of sudden death in the first year after myocardial infarction, decreases the incidence of arteriovenous shunt thrombosis in patients undergoing chronic hemodialysis, and when combined with anticoagulants, may be effective in reducing the frequency of episodes in recurrent venous thrombosis.

Anticoagulants↗
PubMed1980

Late sequelae of splenectomy for trauma.

Hematologic and immunologic changes following splenectomy for trauma include abnormal RBC morphology, leukocytosis, thrombocytosis, impaired clearance of blood-borne particulate antigens, and reduced antibody formation. These abnormalities may be the bases for overwhelming bacterial infection, the most serious delayed complication of splenectomy for trauma. The state of knowledge at present indicates that splenectomized patients should receive pneumococcal vaccination, but whether penicillin prophylaxis should be given daily in addition is less clear. However, penicillin may be advisable for young children, particularly those under 2 years of age, in whom the efficacy of pneumococcal vaccine has not been established. To prevent sepsis, various surgical alternatives to total splenectomy have been proposed, including deliberate autotransplantation of splenic tissue at the time of splenectomy.

Adult↗
PubMed1986

Early rheumatoid arthritis. Approach to diagnosis and treatment.

Early rheumatoid arthritis is a diagnosis most often made by history and physical examination and is frequently a diagnosis of exclusion. Differential diagnosis can be difficult because of the wide variation in age, sex, constitutional symptoms, physical findings, and joint distribution. Early, aggressive therapy should include patient education, rest, graded exercise, counseling, and appropriate medication. Absence of subcutaneous nodules and joint erosion may indicate a better prognosis in rheumatoid arthritis. Presence of rheumatoid factor, eosinophilia, thrombocytosis, and/or vasculitis suggests a less favorable course.

Adult↗
PubMed1993

Kawasaki disease in a Sudanese family.

Kawasaki disease (mucocutaneous lymph node syndrome) is an acute inflammatory multisystem disease of children. The acute phase of the disease is characterized by high grade fever, conjunctivitis, exanthematous skin rash and non-suppurative lymph node enlargement. The subacute phase follows with the manifestations of arthritis, myocarditis and thrombocytosis. The disease is self-limiting in most children but is associated with coronary artery aneurysms in 15-20% of cases. The aetiology is unknown, but results of epidemiological studies suggest that an unidentified infectious agent might be the causative factor. Since the first description of the disease by the Japanese doctor, Tomisaku Kawasaki, in 1967 and his report for the English literature in 1974, thousands of cases have been reported worldwide. The highest prevalence is found in Japan and among the Japanese in Hawaii, followed by the United States. Although the disease was first reported in Africa in 1979, to date only four cases have been reported there. The following account reviews the literature and describes the manifestations of Kawasaki disease as seen in two siblings in Khartoum, Sudan.

Child, Preschool↗
PubMed1998

Mediastinal lymphadenopathy: a variant of incomplete Kawasaki disease.

A 14-month-old girl presented with a 4-d history of fever and generalized exanthema. Four characteristic symptoms of incomplete Kawasaki disease (KD) were present on admission (fever, rash, non-purulent conjunctival injection, oropharyngeal changes) and then followed by oedema of the hands and feet and mild plantar desquamation. The typical laboratory features of KD, such as elevated erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and positive C-reactive protein were also seen. Ultrasound examination of the mediastinum revealed the presence of a lymph node, 30 mm in diameter, below the tracheal carina. Thoracic CT scan confirmed the mediastinal lymph node. The patient was treated with aspirin and intravenous gamma-globulin. Ultrasound study of the mediastinum, which was carried out 6 weeks after hospital discharge, showed that the lymph node had disappeared. This case illustrates that lymph nodes other than cervical lymphadenopathy should be sought when the diagnosis of classical or atypical KD is suspected.

Diagnostic Imaging↗
PubMedDate not supplied

Forty-seven children suffering from chronic myeloid leukemia at a center over a 25-year period.

To determine the diagnostic characteristics, blast crisis status, appropriate treatment, overall patient survival, and prognostic factors pertaining to chronic myeloid leukemia (CML) for children, the authors reviewed 47 children under 18 years of age with CML who were admitted to the National Taiwan University Hospital in the period between 1976 and 2001 inclusively. Between 1976 and 1985, ten patients were diagnosed by clinical manifestation without the application of any cytogenetic study, while from 1986 to 2001, 37 patients were diagnosed as evidenced by the presence of a positive Philadelphia chromosome upon cytogenetic examination. The male-to-female ratio was 0.7 (14 boys/20 girls) for patients under 15 years of age. Abdominal fullness or pain appeared to be the most common presenting symptom, followed by body weight decline and bleeding. Marked leukocytosis, anemia, and thrombocytosis were common laboratory findings in these patients. The incidence of only lymphoid blast crisis was 38%, which appears to be the highest incidence when compared to the corresponding value as reported in other studies. Of 25 children treated with conventional chemotherapy including busulfan and hydroxyurea, the median survival period from diagnosis was 44 and 42 months, respectively. Between January 1984 and December 2001, 22 affected children underwent allogeneic stem-cell transplantation (SCT). The 5-year survival probability following hematopoietic SCT from matched sibling donors is 65%, and 22% following SCT from matched unrelated donors. This difference is statistically significant (p =.01).

Adolescent↗
PubMed2003

Induction of T-cell tolerance in a patient with idiopathic thrombocytopenic purpura by single injection of humanized monoclonal antibody to CD40 ligand.

Normal B cells can be induced to express immune costimulatory molecules by activated T cells, and activated CD4 T cells can express CD40 ligand, a molecule that can engage CD40 on the B-cell surface. CD40-CD40 ligand interaction plays an important role in the pathology of certain autoimmune diseases. We report a patient with chronic idiopathic thrombocyopenic purpura (ITP) who was effectively treated with a single injection of humanized monoclonal antibody to CD40 ligand (E6040). The patient was refractory to steroid therapy, and had baseline platelet counts below 30 x 10(9)/l during the 3-month period before antibody treatment. The patient's platelet counts have increased to more than 100 x 10(9)/l long-term after E6040 administration. Platelet-associated IgG was decreased with thrombocytosis. Compared with the initial period of E6040 administration, the number of anti-GPIIb/IIIa antibody-producing B cells decreased, and proliferative response of autoreactive T cells to GPIIb/IIIa was also improved. A single injection of humanized monoclonal antibody to CD40 ligand may induce T-cell tolerance in patients with ITP.

Antibodies, Monoclonal↗
PubMed2004

Abnormal vascular, platelet and coagulation markers in primary thrombocythaemia are not reversed by treatments that reduce the platelet count.

Although the primary aim of the treatment of primary thrombocythaemia is to reduce platelet counts, its effect on platelet activation, vascular dysfunction and coagulopathy, which are also abnormal, is unknown. We therefore hypothesised that successful treatment of primary thrombocythaemia is accompanied by an improvement in markers of these processes. To test this hypothesis, we compared 38 patients with 15 healthy age and sex-matched controls. Seven untreated patients had platelet counts higher than those 31 on treatment, that were in turn higher than the 15 controls (all p < 0.01). Plasma fibrinogen, P-selectin, von Willebrand factor and sVCAM were higher in both patient groups compared to controls (all p < 0.05), but not between patient groups. sE-selectin and sICAM were unaltered between the three groups. D-dimers were higher in treated patients than in controls. We conclude that successful thrombocytosis treatment does not normalise markers of coagulation, platelet, or endothelial pathophysiology. This may account for continuing risk of pathology in those whose platelet counts have been normalised.

Aged↗
PubMed1997

Correlation between serum gamma-glutamyl transferase activity and the platelet count.

The serum gamma-glutamyl transferase (GGT) activity correlated significantly (P < 0.008) with the platelet count in 55 patients with thrombocytosis. All patients were selected so as to have normal liver function tests(including serum GGT). This correlation was present in both genders and there was no significant correlation with the other liver function tests. These findings are additional evidence that platelets may contribute to the total serum GGT activity.

Journal Article↗
PubMed2002

Essential thrombocythemia (ET): moving from palliation to cure.

Essential thrombocythemia (ET) is a clonal hematopoietic stem cell myeloproliferative disorder characterized by megakaryocytic hyperplasia and persistent thrombocytosis. The clinical presentation and evolution of ET are heterogeneous. This review highlights the current treatment options in the management of ET, including hydroxyurea, anagrelide and both regular and pegylated interferons. Anagrelide, while very effective at controlling counts and symptoms in most patients, may not consistently reduce the bone marrow megakaryocyte mass. Interferon is very effective and not associated with leukemogenesis, but has not been proven to restore polyclonal hematopoiesis and has significant dose-related adverse events. Pegylated interferon represents a significant improvement over the unmodified interferon preparations. Novel therapeutic options directed towards eradication of the malignant ET clone are required.

Female↗
PubMed2002

Thalidomide in myelofibrosis with myeloid metaplasia: a pooled-analysis of individual patient data from five studies.

Trials to determine the effect of thalidomide in patients with Myelofibrosis with Myeloid Metaplasia (MMM) have produced inconclusive results due to different criteria for response and heterogeneous study participants. We undertook a pooled-analysis to assess the effects of such treatment on a larger series of cases and with a uniform assessment of response. We used updated data on 62 individual patients from 5 phase II trials that evaluated thalidomide therapy in MMM patients. Responsewas judged on individual disease parameters, on the improvement of the Dupriez risk categories and on the improvement of a 6 point "severity score" based on myeloproliferative and myelodepletive indexes of the disease. Overall, using standard dose of thalidomide, i.e. starting with no less than 100 mg/day, 49 patients (79%) had more than 4 weeks of therapy. Twenty-nine percent of patients with moderate to severe anemia showed an increase in hemoglobin or reduction/abolishment of blood transfusion requirements, 38% with moderate to severe thrombocytopenia had an increase in platelet counts, and 41% with high grade splenomegaly demonstrated a measurable reduction in splenic size. These effects led to an absolute decrease in the "severity" score in 44.9% of the patients. Major disease severity and high degrees of splenomegaly before therapy predicted response with a probability of 61.9%. However, worsening of the "severity" score was observed in 20.4% of the patients, 18% having a "myeloproliferative reaction" with leukocytosis and/or thrombocytosis. Sixty-six percent of the patients discontinued the drug before 6 months of treatment due to intolerance. In conclusion, there is a small but clear improvement of disease severity with thalidomide therapy in MMM. The potential for myeloproliferative reactions and the unfavorable dose-related toxicity profile argue for future studies using lower doses of this drug.

Adult↗