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PubMed1988

Controlled and uncontrolled thrombocytosis. Its clinical role in essential thrombocythemia.

Only two of 19 patients with spontaneously evolving essential thrombocythemia remained asymptomatic in a 421 patient-month observation. The rest of the patients showed hemorrhagic diathesis (four patients), nonspecific neurological semiology (two patients), and occlusive vascular illness in cerebral, myocardic, arterial, and often multiple locations (total, 12 patients). Peripheral neuropathy was found in five of 10 patients studied. In this series the incidence of cerebral ischemia in the uncontrolled condition was 180 times higher than the epidemiologic expectancy in a population not affected by the disorder. Of 35 ischemic attacks, 22 occurred when the platelet count was more than than 1000 X 10(9)/l, 13 when the count ranged from 650 to 990 X 10(9)/l, and none occurred at counts of less than 650 X 10(9)/l. In contrast, therapeutic control of the thrombocytosis caused all complications to disappear. These findings point out the danger of the natural course of the illness and justify active therapy. At the same time they call into question some of the most commonly used criteria in the diagnosis of essential thrombocythemia.

Adult↗
PubMed1990

Clinical course of essential thrombocythemia in 147 cases.

The authors retrospectively analyzed the initial characteristics, treatment, and clinical course in 147 patients with essential thrombocythemia (ET). Median age was 60 years and the M:F ratio was 0.69. At diagnosis, 53 patients were asymptomatic; 50 patients had functional symptoms (mainly vasomotor disturbances); 27 patients had large vessel thrombosis; 27 patients had a bleeding diathesis; and seven patients had both bleeding and thrombosis. The platelet count ranged from 0.7 to 2.92 X 10(12)/l. Forty-five of the 61 tested patients (61%) had prolonged bleeding time and/or platelet hypoaggregation. Three patients had in vitro spontaneous aggregation. No significant correlations were found between hemostatic findings and in vivo bleeding or thrombosis. The incidence of bleeding, however, was higher in patients with more than 2 x 10(12)/l platelets. Of 87 karyotypes performed with banding techniques, only four were abnormal. One hundred twenty-nine patients received one or more cytoreductive agents at diagnosis or during follow-up. Sixty patients received an antiaggregating agent. First-line therapy was radiophosphorus (32P) in 22 patients; busulfan in 35 patients; and hydroxyurea in 72 patients. Hydroxyurea required continuous maintenance therapy and had to be changed to another treatment in 12 of the initial responders because of inadequate control of thrombocythemia. During follow-up, 14 treated patients experienced one or several major thrombotic events. Two untreated patients also had major thrombosis. Only one major bleeding event was seen during follow-up. Median actuarial survival was 73.5% at 7 years and only one patient progressed to acute non-lymphocytic leukemia (ANLL). These results suggest that large vessel thrombosis is the main complication of ET. It appears largely unpredictable in a given patient at diagnosis but can be largely prevented by the control of thrombocythemia. Because of the low incidence of side effects of treatment in this experience, the authors believe that cytoreductive therapy is indicated in most patients with ET, as long as a group of patients with very low risk of thrombosis is not defined in prospective studies.

Adolescent↗
PubMed1991

Unusual presentation of extranodal peripheral T-cell lymphomas with multiple paraneoplastic features.

Three patients with extranodal peripheral T-cell lymphoma and a distinctive clinical presentation are described. They had acute onset of fever, weight loss, progressive liver failure, bleeding diathesis, pancytopenia, and myelodysplastic changes in the bone marrow. Each patient had one or more paraneoplastic complications: severe rhabdomyolysis with myoglobinuria and secondary renal failure, cutaneous vasculitis, gluten-sensitive enteropathy, polyserositis, and increased macrophages with hemophagocytic activity. They did not have peripheral lymphadenopathy. The complex clinical presentations simulated collagen vascular disorders, systemic infections, or severe liver disease rather than a malignant lymphoma. Routine histologic studies revealed a small population of lymphoma cells in the bone marrow, spleen, and liver. Immunophenotyping studies demonstrated their T-cell phenotype, and cytogenetic analysis showed the clonality in Patients 1 and 2; clonal T-cell receptor gene rearrangement was found in Patients 2 and 3. These studies should be considered in the evaluation of patients with constitutional symptoms, liver failure, coagulopathy, and pancytopenia even in the absence of peripheral lymphadenopathy.

Adult↗
PubMed1992

Ischemic heart disease precipitated by occult cancer.

The hypothesis was tested that cancer, by virtue of it being a thrombotic diathesis, may enhance ischemic heart disease. Because cancer therapy may precipitate thromboembolism, the authors focused on patients with occult neoplasia before therapy. Occult cancer was defined as a time period of 2 years before a cancer diagnosis was established. Data obtained from the files of patients with the diagnosis of malignant tumors and admitted to a general hospital during a 3-year period were reviewed for coronary risk factors, coronary events, and characteristics of cancer. Indices of coronary instability were studied: the incidence of first coronary events, the incidence of all coronary events, and the coronary events burden. These indices were calculated for 366 patients with cancer (from the Department of Medicine files of 166 consecutive patients with cancer of several primary sites and from the surgical ward files of 100 consecutive patients with colorectal cancer and 100 consecutive patients with cancer of the prostate or bladder) and for 100 patients with benign prostatic hypertrophy. The patients with benign prostatic hypertrophy served as controls. A steep and statistically significant increase in coronary instability indices was observed in all groups of patients with cancer in the 2-year period before cancer diagnosis in comparison with the coronary instability indices of control patients (P less than or equal to 0.05 to 0.0001). Patients with colorectal cancer presented the highest indices in the 2-year period before cancer diagnosis, with unstable ischemic heart disease being reported in 18% and first coronary events in 10%. The coronary events burden was 0.92. The lowest indices among patients with cancer were recorded in those with prostatic and bladder cancer. Unstable ischemic heart disease occurred in 6% and first coronary events in 4%. The coronary events burden was 0.36. The indices were several times lower for control patients than for patients with cancer. Unstable ischemic heart disease occurred in 3% of control patients, and first coronary events in 2%. The coronary events burden was 0.15. Other possible etiologic factors, particularly the known coronary risk factors and anemia, were not statistically related with an increased risk of coronary events in the 2-year period before cancer diagnosis. Based on these epidemiologic data, it appears that there may be an association between occult cancer and coronary events.

Adult↗
PubMed2000

Diagnosis of recurrent uterine carcinosarcoma by fine-needle aspiration cytology: report of a case.

Uterine carcinosarcomas are uncommon, aggressive neoplasms usually afflicting postmenopausal women. Histologically, they are characterized by the presence of both malignant epithelial and stromal cells. The latter may be either homologous or heterologous in nature. The cytologic diagnosis of carcinosarcoma via fine-needle aspiration (FNA) was previously described for primary tumors arising in the parotid gland, breast, lung, and ovary. Although the diagnosis of uterine carcinosarcoma via cervicovaginal, endometrial, and peritoneal fluid cytology has been described, the FNA cytology diagnosis of recurrent uterine carcinosarcoma has only been rarely described. We present a case of recurrent uterine carcinosarcoma in a 59-yr-old woman, diagnosed by ultrasound-guided FNA cytology. Cytologic smears showed a biphasic neoplasm consisting of both malignant columnar epithelial and spindle cells, in a background of tumor diathesis. This case illustrates the diagnostic utility of aspiration cytology in the investigation of recurrent carcinosarcoma in clinically suspicious lesions arising postsurgery.

Biopsy, Needle↗
PubMed1994

The stability and prediction of depressive mood states in college students.

Objective criteria for predicting dysphoric mood states in college students from measures of stress and cognitive and personality variables were developed and cross-validated. Contrary to Beck's diathesis-stress model of depression, although dysfunctional attitudes, together with the number and impact of life events, self-esteem problems, and level of traditional feminine personality characteristics, predicted concurrent levels of depressive symptoms, initial level of depression was the best predictor of future depression. Cross-validation of the criteria resulted in accuracy rates of 86 to 87%. Implications of these results and some secondary findings were discussed.

Adolescent↗
PubMed1994

A double-blind placebo-controlled comparison of phenelzine and imipramine in the treatment of bulimia in atypical depressives.

Although antidepressants have been found to be superior to placebo in 12 of 14 studies, the relationship between improvement in the depressive diathesis and bulimia is unclear. In this study, the efficacy of placebo, imipramine, and phenelzine is examined in patients comorbid for atypical depression and bulimia. Greater improvement was observed for both depressive and bulimic symptoms with phenelzine than with either imipramine or placebo. Consistent with its poor antidepressant effects in atypical depression, imipramine seemed to have minimal efficacy for the bulimic symptoms of atypical depressives. These data suggest that the presence of bulimia does not alter the treatment response of atypically depressed patients. Furthermore, the data may suggest a link between depression and bulimia in atypical depressives. Demonstrating a statistical difference with a small sample suggests the effect size is robust, however conclusions are limited by a small sample size.

Adult↗
PubMed2004

Prolonged versus short course of indomethacin for the treatment of patent ductus arteriosus in preterm infants.

BACKGROUND: Indomethacin is a prostaglandin inhibitor used to treat patent ductus arteriosus (PDA) in preterm infants. Although indomethacin produces ductal closure in the majority of cases, it is ineffective in up to 40% of patients. Furthermore, the ductus will re-open in up to 35% of infants who initially respond to the drug. A more prolonged course of indomethacin has been studied regarding the potential to achieve higher rates of ductal closure. OBJECTIVES: To determine if a prolonged course of indomethacin (compared to a short course) reduces the rate of treatment failure in preterm infants with PDA without unwanted side-effects. SEARCH STRATEGY: The search included review of personal files, abstracts of conferences, and the following electronic databases: MEDLINE (1966 to April 2003), EMBASE (1974 to April 2003), and Oxford Database of Perinatal Trials, Cochrane Central Register of Controlled Trials (The Cochrane Library, Issue 3, 2003). No language restrictions were applied. SELECTION CRITERIA: 1) DESIGN AND POPULATION: Randomized or quasi-randomized controlled trials including preterm infants with PDA diagnosed on clinical and/or echocardiographic examination.2) INTERVENTION: Indomethacin treatment by any route given as a long course (four or more doses) vs a short course (three or fewer doses). 3) OUTCOMES: Report of at least one of the following outcomes: failure of PDA to close, need for re-treatment, PDA re-opening, PDA ligation, mortality, duration of assisted ventilation, chronic lung disease (CLD), duration of supplemental oxygen dependence, intraventricular hemorrhage (IVH) (all and severe), diminished urine output, increased serum creatinine, necrotizing enterocolitis (NEC), bleeding diathesis, retinopathy of prematurity (ROP), and duration of hospital stay. DATA COLLECTION AND ANALYSIS: The three reviewers independently abstracted data from each study. Relative risk (RR) and Risk Difference (RD) with 95% confidence intervals (CI) using the fixed effect model for meta-analysis are reported. When a statistically significant RD was found, the number needed to treat (NNT) or number needed to harm (NNH) was also calculated with 95% CIs. A chi-square test was used to test for heterogeneity of results among included trials. MAIN RESULTS: Prolonged indomethacin treatment when compared to the short course resulted in a borderline statistically significant difference in PDA re-opening rate favoring the prolonged course [RR 0.54 (95% CI 0.3, 0.99); RD -0.12 (95% CI -0.24, -0.01); NNT = 8 (4, 100). There was no statistically significant treatment effect on PDA closure, re-treatment, or ligation rates. The prolonged course was associated with a decreased incidence of severe IVH [RR 0.49 (95% CI 0.25, 0.98); RD -0.12 (95% CI -0.24, -0.01); NNT 8 (4, 100)] and renal function impairment, as evidenced by a lower proportion of infants having an increased creatinine level [RR 0.52 (95% CI 0.34, 0.81); RD -0.20 (95% CI -0.33, -0.08); NNT 5 (3, 13)]. However, there was a trend for the prolonged course to increase the proportion of infants with CLD in the one trial reporting this outcome [RR 2.24 (95% CI 0.98, 5.12); RD 0.24 (95% CI 0.01, 0.47)]. REVIEWER'S CONCLUSIONS IMPLICATIONS FOR PRACTICE: Prolonged as compared to short course of indomethacin for the treatment of PDA in preterm infants has a borderline effect on reducing the rate of PDA re-opening and it may be associated with an increased risk for CLD. However, prolonged course of indomethacin appears to reduce the risk of severe intracranial hemorrhage and renal impairment in this population. Definitive recommendations about the preferred duration of indomethacin therapy, i.e. prolonged versus short course, for the treatment of PDA in premature infants cannot be made based on the current findings of this review. IMPLICATIONS FOR RESEARCH: There is a paucity of data on optimal duration of indomethacin therapy for the treatment of PDA, in particular for ELBW premature infants. Future randomized clinical trials should include this high risk population and investigate the premature infants. Future randomized clinical trials should include this high risk population and investigate the possibility of tailoring duration of therapy (prolonged versus short) to individual response in terms of echocardiographic findings and/or prostaglandin levels, focusing on clinically significant outcomes and potential complications associated with either strategy. In addition, factors which may influence treatment effect need to be taken into account when designing such studies.

Ductus Arteriosus, Patent↗
PubMed2001

Bruising and the ketogenic diet: evidence for diet-induced changes in platelet function.

Excessive bruising is a symptom noted by parents of some children treated with the ketogenic diet for epilepsy control, although this side effect is not reported in the literature. We evaluated our cohort of current and past diet-treated patients for symptoms of bruising or bleeding through chart review and prospective screening at clinic follow-up visits. A significant increase in bruising or other minor bleeding was reported and/or observed in 16 of 51 patients (31.4%). There were no differences in sex distribution or number of anticonvulsants used between patients with bruising/bleeding and those without this symptom, although the group with bruising/bleeding was significantly younger. No specific anticonvulsant was associated with bruising/bleeding. Six patients with diet-induced bruising/bleeding underwent an investigation for bleeding diathesis. Five of these patients had prolonged bleeding times and all had diminished responsiveness to various platelet aggregating agents, with no evidence of a release defect. The abnormalities all normalized in the 1 patient tested after ceasing the diet. No patients had serious hemorrhage. One patient had mild von Willebrand disease, which had been asymptomatic before diet initiation. Some patients were Stimate responsive, suggesting a treatment for more severe bouts of symptoms. These data suggest that a ketogenic diet-related bleeding tendency occurs in about one third of treated patients owing to preexisting factors defining susceptibility in combination with diet-induced depression of platelet responsiveness, possibly related to changes in platelet membrane lipid composition and/or concentration and resultant effects on function of membrane-embedded proteins. Patients on the diet undergoing anticoagulation or surgery should be evaluated carefully for symptoms of bleeding tendency.

Blood Platelets↗
PubMed2004

C1272S: a new candidate mutation in type 2A von Willebrand disease that disrupts the disulfide loop responsible for the interaction of VWF with platelet GP Ib-IX.

Most of type 2A von Willebrand disease (VWD) mutations are clustered within the A2 domain of VWF, encoded by the 3' region of exon 28 of the von Willebrand factor (VWF) gene. A patient with lifelong and severe bleeding diathesis and laboratory data of type 2A VWD is described. The analysis of the complete exon 28 of the VWF gene showed a 3815 G-->C change within the A1 domain, resulting in the C1272S missense mutation in a heterozygous state. The substitution was not found in 100 normal alleles also examined and has not been described previously. This candidate mutation would interrupt the formation of the disulfide loop 1272-1458, which is important in maintaining the adequate conformation of the VWF functional domain that interacts with platelet glycoprotein Ib-IX. Gene expression of this candidate mutation is necessary to confirm its role.

Adult↗
PubMed2004

Russia's imperial blood: was Rasputin not the healer of legend?

The only son of Russia's last Tsar, a great-grandson of Queen Victoria, continues to be used as the favorite example of the X-linked inheritance of hemophilia, in spite of the fact that this popular historical diathesis has never been confirmed by any form of modern medical laboratory testing. Certain to be controversial, a new study of the symptoms that were witnessed by those who were closest to the teenaged Russian heir now raises the possibility that his blood disorder might well have been something other than hemophilia. The key to discovering Tsarevich Alexei's true diagnosis is found in those now legendary allegations that the infamous "Mad Monk", Grigory Rasputin, had possessed a power of healing that was somehow responsible for the young boy's mysterious history of spontaneous recoveries. If we are to accept the popular diagnosis of history and call it a clotting factor deficiency, then the boy's now famous sudden recoveries will remain a complete mystery. The so-called "Mad Monk" Rasputin, as a direct result of the revolutionary propaganda of the time, is then overblown into a larger-than-life legend. If, however, we are to change the diagnosis and call it a platelet disorder, then the air is let out of the legend, and Rasputin is revealed to have been nothing more than a very ordinary middle-aged Siberian hippie who did not possess any healing powers at all.

Anemia, Hemolytic↗
PubMed1978

Studies on a new variant of the Hermansky-Pudlak syndrome: qualitative, ultrastructural, and functional abnormalities of the platelet-dense bodies associated with a phospholipase A defect.

The structure and functions of platelets from a patient in whom albinism and hemorrhagic diathesis were associated have been investigated. Electron microscope studies showed a large reduction in the number of dense bodies and this was confirmed by an examination of fluorescent platelets loaded with mepacrine. The rare dense bodies were much bigger than normally observed; their density was diminished and was localized in a peripheral ring. Other platelet constituents were found to be normal. Platelet peroxidase activity was normal in the canaliculi of the dense tubular system; catalase-positive granules were also present. Serotonin uptake by the patient's platelets was much decreased and reserpine, a potent inhibitor of serotonin accumulation by normal human platelets, did not further decrease this incorporation. The uptake of free 14 C-arachidonic acid by the platelets was greatly diminished, as was its thrombin-induced liberation from phosphatidyl-choline and phosphatidyl inositol. Moreover, platelet phospholipase A1 activity was much reduced and phospholipase A2 activity was undetectable.

Adult↗
PubMed1979

Bleeding time in uremia: a useful test to assess clinical bleeding.

Modified Ivy bleeding time (template) and platelet aggregation to ADP, epinephrine, and collagen were studied in 26 uremic patients who had not recently ingested anti-platelet drugs. Regardless of the aggregating agent used, the abnormalities in platelet aggregation were often mild, even with advanced uremia, and frequently less severe than the effects of common anti-platelet drugs. The inhibition of collagen-induced aggregation was significantly correlated with both increased bleeding time and blood urea nitrogen. Platelet aggregation was not discriminative between clinically bleeding and non-bleeding groups of patients, but the bleeding time was helpful in this regard. In certain cases, the aggregometric patterns differed between drug-induced and uremic thrombocytopathies. Platelet aggregometry appears to be of little help clinically in assessing the severity of the uremic bleeding diathesis.

Adenosine Diphosphate↗
PubMed1979

Acute promyelocytic leukemia.

Acute promyelocytic leukemia (APL) is characterized by proliferation of morphologically abnormal promyelocytes and a severe bleeding diathesis. The abnormal promyelocyte is characterized by abundant, large granules, many of which are spindle-shaped. Electron microscopic appearance of the granules closely resembles that of Auer rods. The granules appear to possess tissue thromboplastin activity by both immunologic and clotting assays. Coagulation studies in APL are generally consistent with disseminated intravascular coagulation. Prolongation of the prothrombin time and elevation of fibrinogen degradation products are the tests that are most commonly abnormal. Although occasional reports indicate a favorable response of the coagulopathy to drugs that inhibit fibrinolysis, the use of prophylactic heparin appears to be the treatment of choice. The response rate of APL to chemotherapy regimens that contain an anthracycline is comparable to that of acute myelogenous leukemia. The recent description of the 15;17 chromosomal translocation which may be pathognomonic for APL is only the second example of a chromosomal marker of human neoplasia. Marked elevation of serum vitamin B12 and B12 binding proteins appears to be another characteristic feature of APL. An in vitro cell line of APL cells has been demonstrated to have the capacity to differentiate to functional polymorphonuclear leukocytes, but the cause for the maturation arrest is unknown.

Blood Coagulation Disorders↗
PubMed1985

Transient Passovoy defect during a febrile illness.

The Passovoy defect is a recently characterized hemorrhagic diathesis. We describe a patient with a febrile illness, possibly from Epstein-Barr (EB) virus, who acquired this defect transiently. Prothrombin time; assays for factors VIII, IX, XI, XII; and Fletcher (prekallikrein) and Fitzgerald (high molecular weight kininogen) factors were normal. No definite circulating inhibitor could be demonstrated. The transient Passovoy defect could possibly be ascribed to the infectious process or sulfisoxazole, which the patient had received.

Adult↗
PubMed1987

Fibrinogen giessen I: a congenital homozygously expressed dysfibrinogenemia with A alpha 16 Arg----His substitution.

Clinical reports are published for only two patients with homozygously expressed congenital dysfibrinogenemia. The patients, both of whom have a bleeding diathesis, have amino acid substitutions in the fibrinogen molecule at A alpha 16 Arg----Cys and A alpha 19 Arg----Ser, respectively. We report that a third patient with dysfibrinogenemia (fibrinogen Giessen I) is homozygous for A alpha 16 Arg----His. Although this patient has had excessive postpartum bleeding, she has had normal hemostasis throughout several minor surgical procedures and hysterectomy. Elucidation of the amino acid alterations in patients with dysfibrinogenemia may expand our understanding of structural determinants of fibrinogen that are critical to its function in vivo.

Adult↗
PubMed1987

Reliability of absent platelet dense bodies as a diagnostic criterion for Hermansky-Pudlak syndrome.

The clinical, pigmentary, and ceroid storage manifestations of the Hermansky-Pudlak syndrome (HPS) triad of albinism, hemorrhagic diathesis, and ceroid storage disease are variable. Therefore, a rapid and accurate method of diagnosing HPS is needed. Platelets of 66 albinos were examined by electron microscopy for the presence or absence of dense bodies. Results show that patients reexamined over a period of 1 year had consistent findings. Those lacking dense bodies (15) when first examined also lacked dense bodies when reexamined a year later, and they had evidence of ceroid storage. Those with dense bodies when first examined (8) also had dense bodies when reexamined, did not have evidence of storage disease, and had types of albinism other than HPS. Of 20 propositi lacking dense bodies, all 32 albino relatives also lacked dense bodies, while 6 albino relatives of 6 propositi with dense bodies also had dense bodies in their platelets. The evidence supports the concept that HPS is a distinct genetic and biochemical disease in which the components of the triad are the result of a single genetic defect, either a point mutation or a small deletion. Comparison of whole mount preparations with thin section preparations of 13 albinos shows that whole mount preparations are an accurate and rapid method for diagnosing HPS. The most consistent diagnostic feature of HPS is lack of platelet dense bodies.

Albinism↗
PubMed1988

Thrombosis following desmopressin for uremic bleeding.

An elderly patient with evidence of atherosclerosis and uremic bleeding diathesis developed two foci of cerebral thrombosis immediately after an infusion of desmopressin (DDAVP). Because large molecular weight multimers of von Willebrand factor (vWF) have been demonstrated to cause platelet aggregation under conditions of elevated fluid shear stress as occurs in atherosclerotic vessels, we investigated his plasma vWF at the time of the event and compared it to baseline values obtained 2 weeks later. Unusually large vWF multimers induced by the DDAVP infusion were present and likely contributed to the thrombotic process. Consequently, we believe DDAVP should be given with greater caution to patients with atherosclerosis.

Aged↗