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At least 433 records · Page 24Linked to original sources
PubMed2006

Inflammatory myofibroblastic tumor with thrombocytosis and a unique chromosomal translocation With ALK rearrangement.

We describe a case of inflammatory myofibroblastic tumor with an unusual constellation of clinical, pathologic, and genetic findings. A 7-year-old girl had an 11-cm abdominopelvic mass accompanied by thrombocytosis, anemia, elevated erythrocyte sedimentation rate, and elevated C-reactive protein. The inflammatory myofibroblastic tumor displayed unusual histologic features of zonal coagulative necrosis, high cellularity with a herringbone pattern, and tumor-associated osteoclast-like giant cells. The complex tumor karyotype included a translocation t(1;2)(q21; p23). Following resection, the laboratory abnormalities resolved. The patient is well and free of recurrence at 3 years following resection. This case raises interesting questions about clinical, pathologic, prognostic, and molecular genetic interrelationships in inflammatory myofibroblastic tumor.

Abdominal Neoplasms↗
PubMed1996

Leustroduscin B, a new cytokine inducer derived from an actinomycetes, induces thrombocytosis in mice.

Leustroduscin B (LSN-B), a novel colony-stimulating factor (CSF) inducer produced by an actinomycetes, has previously been shown to induce CSF production in bone marrow stromal cells. To determine the biological activity of LSN-B on hematopoiesis in vivo, LSN-B was administered intraperitoneally to mice every day for three to six days. Peripheral platelet counts were markedly elevated on days 4 through 6 compared with the control mice injected with vehicle. Serum II/6 levels were low (0.8 ng/ml) or virtually undetectable in the drug treated groups. This cytokine profile suggests that LSN-B induction of thrombocytosis is mechanistically distinct from other cytokine inducers such as IL-1 or FK-565.

Animals↗
PubMed1990

Multicentric angiofollicular lymph node hyperplasia with peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis in women. A distinct syndrome.

Four women with multicentric angiofollicular lymph node hyperplasia had a distinct clinical syndrome characterized by peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis. The nodes displayed typical morphologic changes of the plasma cell variant of multicentric angiofollicular lymph node hyperplasia. The pathologic changes are morphologically distinct from angioimmunoblastic lymphadenopathy with dysproteinemia although clinical similarities do exist. In these four cases, the lymphadenopathy was usually bulky and multicentric. There was frequent splenic involvement. The neuropathies were severe and disabling. Clinical courses have been variable with some responses to therapy with steroids and alkylating agents. No neoplastic transformations have occurred. Multicentric angiofollicular lymph node hyperplasia may represent a reactive lesion in which the antigenic stimulus is unknown but results in follicular hyperplasia, angiogenesis, and the systemic manifestations of hyperimmune stimulation. We believe this clinical syndrome may represent a distinct variant of multicentric angiofollicular lymph node hyperplasia, and it requires close observation for neoplastic transformation and other complications of its multisystem nature.

Biopsy↗
PubMed1976

[Studies on the problem of septic thrombocytopenia and reactive thrombocytosis in obstetrical patients].

During the first 24 hours of septicaemia the platelet count decreases, but in about a week increases again resulting a significant reactive thrombocytose. According to the results of platelet spreading test, the thrombopenia is caused by consumption of young platelets. During the course of increase of platelet count the number of haemostatically most active, newly formed platelets exceeds, and this is why in the periode of reactive thrombocytosis deep-vein thromboses may occure against Heparin or oral anticoagulant treatment.

Bacterial Infections↗
PubMed1999

Longitudinal melanonychia associated with hydroxyurea therapy in a patient with essential thrombocytosis.

Occurrence of skin lesions during long-term therapy with hydroxyurea are well described, but there have been only a few case reports of longitudinal melanonychia associated with hydroxyurea treatment. We report herein longitudinal melanonychia associated with hydroxyurea therapy in a subject with essential thrombocytosis. The prolonged time lapse (1 year) between initiation of hydroxyurea therapy and the onset of the longitudinal melanonychia should be noted.

Aged↗
PubMed2001

[Chronic progressive polycythemia and thrombocytosis].

The 73-year-old female patient presented with chronic progressive erythrocytosis diagnosed 5 years ago which now is accompanied by thrombocytosis. She complains about generalised itching, pain in distal limbs and shows marked plethora. Having excluded major causes of secondary erythrocytosis the diagnosis was polycythaemia vera with a typical constellation of symptoms, findings and course of disease. Under treatment with 5-Hydroxyurea and repeated phlebotomy thrombocyte count and haematocrit normalised and the patient recovered her usual vitality. Limb pain, itching and plethora disappeared.

Aged↗
PubMed2002

Leflunomide induced fevers, thrombocytosis, and leukocytosis in a patient with relapsing polychondritis.

The most common adverse events reported with the use of leflunomide are hypertension, infections, alopecia, and various gastrointestinal complaints. No fatal adverse hematologic events have been reported in humans, although anemia and leukopenia have been described in animals receiving 20 mg/kg/day. We describe a patient with relapsing polychondritis, in whom treatment failure with glucocorticoids, methotrexate, hydroxychloroquine, and azathioprine led to the institution of therapy with leflunomide at a maintenance dose of 20 mg/day. Two months after the dose of leflunomide had been increased to 30 mg daily, the patient developed high fevers, photophobia, thrombocytosis, and leukocytosis that returned to normal following treatment with cholestyramine and discontinuation of leflunomide. Rechallenge with leflunomide was not attempted and the syndrome did not recur during 14 month followup.

Anti-Inflammatory Agents↗
PubMed2002

Thrombocytosis: too much of a good thing?

Thrombocytosis is due to (a) a variety of disorders that cause reactive stimulation of platelet production, (b) familial mutations, or (c) essential thrombocythemia (ET) and other myeloproliferative disorders (MPDs). The MPDs are clonal abnormalities of the pluripotent hematopoietic stem cell. Dysregulation of megakaryocytopoiesis in ET involves defective binding of thrombopoietin by platelets and megakaryocytes resulting in increased levels of plasma free thrombopoietin, and increased sensitivity of megakaryocytes to thrombopoietin leading to their increased proliferation. Bleeding and thrombosis are the major causes of morbidity and mortality in ET and the other MPDs. The elevated platelet count and qualitative platelet defects have been implicated in the pathophysiology of these hemostatic problems. However, these platelet abnormalities do not correlate well with clinical complications. It is proposed that bleeding and thrombosis could be due to vascular abnormalities that result from dysfunctional hematopoietic stem cell-derived endothelial cells.

Blood Platelets↗
PubMed2002

[Pregnancy-related thrombocytosis].

Pregnancy-related thrombocythaemia comprises myeloproliferative and inflammatory reactive subsets. In pregnant women treated for myeloproliferative disorders, especially polycythaemia vera and primary thrombocytosis, only 50-70 per cent are delivered successfully of a normal healthy baby. The maternal complications are cerebral, cardiac, and abdominal arterial thrombosis, and with deep venous thrombosis of the legs, whereas bleedings are mainly seen in the case of extreme thrombocythaemia, owing to absorption of factors by the platelets. The foetal complication are dominated by abruptio placentae, pre-eclampsia, placental insufficiency, and death. Reactive thrombocythaemia includes the physiological rise in platelets postpartum, believed to be part of the normal maternal haemostasis, which almost never causes thromboembolic complications, as far as is known today. In contrast, the inflammatory reactive thrombocythaemia, related to severe foetal and/or maternal necrosis, is generally related only to a moderate rise in the platelet count. As the blood-platelet count does not appear to be routine at general pregnancy check-ups, it is necessary to be aware of risk groups, consisting of women with otherwise unexplained abortions or stillbirths, unexplained foetal and placental malformations, and pre-eclampsia, even if the woman has never had any thromboembolic complications.

Anticoagulants↗
PubMed1992

Establishment of large cell lung cancer cell lines secreting hematopoietic factors inducing leukocytosis and thrombocytosis.

We have established cell lines from a large cell carcinoma of the lung accompanied by marked granulocytosis and thrombocytosis, and have analyzed the factors with colony stimulating factor (CSF) activity produced by them. Analysis of the CSF activity present in the culture medium of the established cell lines demonstrated growth-stimulating activity on CMK cells, a human megakaryoblastic cell line and mouse bone marrow cells. A neutralization test with antibodies against G-, M- and GM-CSF indicated the stimulation for the proliferation of CMK and mouse bone marrow cells to be mediated partially by the CSFs. Furthermore, the measurement of GM-CSF and interleukin(IL)6 by enzyme-linked immunosorbent assay (ELISA) and northern blotting analysis indicated productions of G-, GM- and M-CSF and of IL6 from the cell lines but failed to exhibit IL3 gene expression. It is suggested that the cell lines could be of use in the study of CSFs and, also, that lymphokines act on leukocyte and platelet progenitor cells.

Adult↗
PubMed2003

Anagrelide-induced bone marrow changes during therapy of chronic myeloproliferative disorders with thrombocytosis. an immunohistochemical and morphometric study of sequential trephine biopsies.

BACKGROUND AND OBJECTIVES: Anagrelide is an agent with a significant platelet-lowering activity in humans. Contrasting the wealth of clinical data, bone marrow (BM) changes during therapy have been relatively rarely studied; information is particularly lacking regarding specific features of megakaryocytopoiesis. DESIGN AND METHODS: A study was performed on 15 patients with essential thrombocythemia and early stage chronic idiopathic myelofibrosis presenting with an elevated platelet count. These patients received anagrelide for 25 months resulting in a significant improvement of thrombocytosis. Evaluations were carried out by morphometry on sequential BM biopsies following enzyme- and immunohistochemical stainings that also included those for proliferative capacity and apoptosis. RESULTS: No significant change in proliferation or apoptosis was recognizable during anagrelide treatment. The most conspicuous alterations were those of the CD61+ megakaryocytopoiesis. Megakaryocytopoiesis revealed an increase in promegakaryoblasts together with an enhancement of proliferating cell nuclear antigen activity. This feature is in keeping with an inhibitory effect on endoreduplication implying an arrest of polyploidization and maturation into platelet-shedding large megakaryocytes. On the other hand, a significant increase in the number of megakaryocytes was not detectable. Anagrelide failed to exert a stimulating influence on the progression of myelofibrosis or on the amount of CD34+ progenitor cells. Regarding angiogenesis, there was no increase in the density of BM vessels, but distension of the vascular lumina corresponded with a vasodilatory effect. INTERPRETATION AND CONCLUSIONS: Anagrelide exerts a significant effect on endoreduplicative activity of megakaryocytes consistent with an inhibition of maturation and therefore, generates a relative predominance of precursor cells but fails to stimulate myelofibrosis.

Adult↗
PubMedDate not supplied

[Recurring ischemic cerebral infarction caused by primary thrombocytosis in infancy].

INTRODUCTION: Ischemic cerebrovascular disease includes a set of entities that are produced by disorders in components of the blood, the blood flow, the walls of blood vessels or the heart, and can be anatomical, functional or even mixed. CASE REPORT: We describe the case of an 18-month-old male patient with compensated celiac disease, with repeated ischemic strokes in different territories, including the right posterior cerebral artery and middle cerebral artery, in the course of a hypercoagulable state due to essential thrombocytosis. Computerised axial tomography scans, brain angioresonance, a complete blood chemistry analysis and bone marrow biopsy were all performed and confirmed the previous diagnosis. Exchange transfusion, antiplatelet drugs and a platelet production inhibitor (anagrelide) were begun as therapy. At present, the patient is 2 years old and still has a slight direct hemiparesis, which is complete and predominantly faciobrachial, with no alterations to language. CONCLUSIONS: Cerebral infarctions in infancy are infrequent, and their presentation obliges the attending clinician to seek causes that are not usual. In our patient the hypocoagulability came about due to essential thrombosis, which is rare in infancy. The cause behind the infarction determines the chances of its recurring. Acetylsalicylic acid did not prove to be effective for this purpose. We suggest using carbamazepine for the treatment of kinesigenic dystonias.

Brain Ischemia↗
PubMed2006

Essential thrombocytosis and myocardial infarction in an aircrew member: aeromedical concerns.

Of essential thrombocytosis (ET) cases, 25% occur in patients younger than 40 yr of age, and are often discovered as an incidental laboratory abnormality. However, the risk for thrombosis remains of concern and needs to be closely evaluated, especially in the aerospace environment. We report on the case of a 40-yr-old, female French military air traffic controller (ATC) admitted for an ST-elevation myocardial infarction. She was a smoker and had no previous medical history of ET. The coronary angiogram showed a thrombus of the left anterior descending coronary artery. She was treated medically with angioplasty and stent. Laboratory data revealed an elevated platelet count (495,000 x mm(-3)), confirmed 6 mo later (645,000 x mm(-3)). The diagnosis of ET was then established. No platelet-lowering therapy was prescribed, aspirin was continued, and this ATC was considered unfit for operational duties. Arterial thrombosis is more frequent than venous in ET, and can affect the whole arterial tree from the microscopic to the main arteries. Thrombosis is unpredictable and, due to abnormalities of the platelet functions and associated cardiovascular risk cofactors, may occur even with an almost normal platelet count. Risk-adjusted therapy is needed, including lifestyle modification to address vascular risk factors, antiplatelet drugs (aspirin), and platelet-lowering agents with their risk of leukomutagenesis. Furthermore, there is no consensus for the prevention of venous thrombosis. The decision for the aeromedical expert is difficult and depends on the specialty of the aircrew member, the type and duration of the mission, the therapeutics used, and the benefit-risk ratio of platelet-lowering agents.

Adult↗
PubMed2006

Occurrence of the JAK2 V617F mutation in the WHO provisional entity: myelodysplastic/myeloproliferative disease, unclassifiable-refractory anemia with ringed sideroblasts associated with marked thrombocytosis.

The JAK2/V617F mutation has been noted in essential thrombocytemia. We investigated 19 cases with refractory anemia with ringed sideroblasts (RARS), including three RARS with thrombocytosis (RARS-T). Only the RARS-T patients showed this mutation. More cases need to be analyzed to determine the prevalence of the JAK2/V617F mutation in RARS-T.

Aged↗
PubMed2006

[Acute spinal epidural hematoma complicated with essential thrombocytosis].

An 82-year-old woman was brought to our hospital with back pain and paraplegia. Her platelet level was elevated to 456 x 10(4) x mm(-3). She was diagnosed as having acute spinal epidural hematoma complicated with essential thrombocytosis. After emergency decompression surgery, chemotherapy with hydroxycarbamide was performed and platelets decreased to 80 x 10(4) x mm(-3). There were no perioperative complications, such as hemorrhage or thrombosis in other organs.

Aged, 80 and over↗
PubMed1991

Dysmegakaryocytopoiesis and thrombocytosis in a patient with acute myelomonocytic leukemia and long evolution.

A patient with acute myelomonocytic leukemia (M4), dysmegakaryocytopoiesis and thrombocytosis is presented. Immunophenotyping and blast colony assay showed the presence of blasts with IaDr, CD 33 and CD 14 antigens. Cytogenetic analysis and level of thrombopoietin were normal. This atypical case represents yet another example of the transitional zone between atypical myeloproliferative disease and acute leukemia, and the apparent absence of any cytogenetic abnormality is noteworthy.

Humans↗
PubMed1990

Thrombocytosis and thrombocythemia.

Elevated platelet counts may be encountered as a reactive phenomenon secondary to a variety of systemic conditions (thrombocytosis) or may represent a primary disorder of the bone marrow (thrombocythemia). The diagnosis of essential thrombocythemia is difficult and relies on exclusion of other myeloproliferative states and nonhematologic illnesses associated with increased platelet number. The paradoxic clinical complications of hemorrhage and thrombosis, the presence of splenomegaly, and the finding of various qualitative platelet abnormalities point to existence of the neoplastic disorder. Although treatment of the symptomatic patient with platelet lowering agents or antiplatelet drugs may be indicated and effective, the role of therapy in the asymptomatic individual remains highly controversial.

Adolescent↗
PubMedDate not supplied

[Thrombocytosis: general frequency and etiological circumstances (author's transl)].

During a period of five years, fifteen thousand platelet counts has been systematically realised at the Hôtel-Dieu Hospital in Marseilles. A thrombocytosis superior to 400 000/mm3 has been observed in 1,2% of cases, in such various etiological cases as mainly cancer (30%), infectious diseases (28%) and post-hemorragic anaemias (11%).

France↗