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PubMed2000

The role of cognitive vulnerability and stress in the prediction of postpartum depressive symptomatology.

OBJECTIVES: The present study was designed to test the diathesis-stress components of Beck's cognitive theory of depression and the reformulated learned helplessness model of depression in the prediction of postpartum depressive symptomatology. DESIGN AND METHODS: The research used a two-wave longitudinal design--data were collected from 65 primiparous women during their third trimester of pregnancy and then 6 weeks after the birth. Cognitive vulnerability and initial depressive symptomatology were assessed at Time 1, whereas stress and postpartum depressive symptomatology were assessed at Time 2. RESULTS: There was some support for the diathesis-stress component of Beck's cognitive theory, to the extent that the negative relationship between both general and maternal-specific dysfunctional attitudes associated with performance evaluation and Time 2 depressive symptomatology was strongest for women who reported high levels of parental stress. In a similar vein, the effects of dysfunctional attitudes (general and maternal-specific) associated with performance evaluation and need for approval (general measure only) on partner ratings of emotional distress were evident only among those women whose infants were rated as being temperamentally difficult. CONCLUSION: There was no support for the diathesis-stress component of the reformulated learned helplessness model of depression; however, there was some support for the diathesis-stress component of Beck's cognitive theory.

Adult↗
PubMed2003

Psychological trauma and psychosis: another reason why people diagnosed schizophrenic must be offered psychological therapies.

This article summarizes the research literature documenting the high prevalence of psychological trauma, including childhood sexual and physical abuse, among people diagnosed psychotic in general and schizophrenic in particular. A review of the relevant literature indicates that childhood trauma may in some cases be causally related to the development of psychotic symptomatology later in life, perhaps by contributing to the diathesis in the stress-diathesis equation. However it is argued that regardless of whether clinicians view the relationship as causal, contributory, co-morbid, or coincidental is irrelevant to the fact that this high prevalence dictates that all people diagnosed schizophrenic receive a proper trauma assessment and, where appropriate, be offered psychological treatments to address the sequelae of the trauma or abuse. Taken in conjunction with the proven effectiveness of various psychological and psychosocial treatments in ameliorating the symptomatology and improving the quality of life of people diagnosed schizophrenic, the need to address the trauma found in such high rates in this population requires a broadening, and not as some suggest a limitation, of the range of treatments offered. The authors identify and challenge ideologically, rather than empirically, driven assumptions about the etiology of schizophrenia, particularly the distorted but dominant version of the diathesis-stress model, which claims that the diathesis is predominantly or exclusively biogenetic in origin and therefore inaccurately places all psychosocial stressors and traumas exclusively on the stress side of the equation. It is this bias that inhibits many clinicians from taking trauma histories with people diagnosed schizophrenic and from offering appropriate treatment when trauma is disclosed. It is the same bias that leads some to argue for a reduction in the availability of psychological therapies for such people.

Adult↗
PubMed2001

[The course of peptic ulcer in relation to patients' constitution].

Sixty one patients with peptic ulcer (PU) were examined to assess the specific features of its condition in relation to their affiliation to the type of morphological constitution (diathesis). Patients with PU were found to have most commonly dyscrasic diathesis. With predominant dyscrasic diathesis, there were more frequently complications of PU (rough scarring deformations of the gastric or duodenal walls, ulcer bleeding, perforation without clear association with the advance of PU, patient compliance, and the adequacy of antiulcerous therapy. Arthritic diathesis was next to dyscrasic one in its prevalence. Other diatheses (tuberculinic, psoriatic) were encountered much less frequently. Analysis of the diatheses manifested in a patient allows one to predict the course of PU, which is determined not only by the activity and duration of conventional antiulcerous treatment, as evidenced by these studies.

Adult↗
PubMed2001

[Mite allergy and neural damage].

The number of adult patients with atopic disorders is increasing in Japan. We recently reported the occurrence of myelitis in patients with atopic diathesis (atopic myelitis). The characteristic features of myelitis with atopic diathesis are: (1) paresthesia/dysesthesia as the predominant symptom, (2) mild and infrequent, if any, muscle weakness, (3) preferential involvement of the cervical cord, (4) persistence of neurologic symptoms and MRI lesions, (5) hyperIgEaemia, (6) mite antigen-specific IgE in serum, and (7) normal cerebrospinal fluid findings. The spinal cord lesions show eosinophilic inflammation on biopsy. We also found an association of Hirayama disease, juvenile muscular atrophy of the distal upper limb, with atopic diathesis. In children, poliomyelitis-like illness following asthma attacks is well known as Hopkins syndrome. These three conditions suggest a link between atopic diathesis and the development of spinal cord disease.

Adult↗
PubMed1991

[Acquired coagulopathy caused by administration of parenteral broad-spectrum antibiotics].

Between October 1988 and May 1989, four cancer patients treated by broad-spectrum antibiotics developed a hemorrhagic diathesis induced by vitamin k (VK) deficiency. Activated partial thromboplastin time (APTT), prothrombin time (PT), factor II (FII) and protein induced by vitamin k absence of antagonist-II (PIVKA-II) were measured after administration of antibiotics and VK in all 4 patients. All these patients had been receiving intravenous hyperalimentation (IVH) and antibiotics for various infections. But all or them developed hemorrhagic diathesis within five days after the initiation of broad-spectrum cephem antibiotics (LMOX or CMNX). The abnormalities were 1) marked decrease of F II (6-18%), 2) prolongation of APTT (58.4-200 seconds), 3) prolongation of PT (7-21%), 4) marked increase of PIVKA-II (17-80 less than AU/ml). After being treated by intravenous administration of VK, hemorrhagic diathesis and abnormalities of coagulation tests except for PIVKA-II were corrected quickly in three evaluated patients. The measurement of PIVKA-II seemed to be useful to diagnose the hemorrhagic diathesis caused by VK deficiency in the patients during administration of antibiotics.

Aged↗
PubMed1995

[Wilson's disease in personal material--disturbances in hemostasis].

Genetically determined impairment of copper excretion from the liver into the bile in Wilson's disease (WD) cause that "free copper" is accumulated in toxic amounts not only in the liver, but also in other organs. In WD liver biopsy often could not be made because of serious disturbances in hemostasis. The aim of the study was: a) to demonstrate our 9 patients with various form of WD. b) to examine some blood clotting factors and compare the results with these obtained in other liver diseases. The diagnosis of Wilson's disease was made on the basis of disturbed copper metabolism. Among our 9 patients (8 women and 1 man, between 17-33 years old) we diagnosed: 3 patients with fulminant Wilson's disease with all day deep jaundice, hemolytic anemia, haemorrhagic diathesis and liver failure, died, 2 patients with active chronic hepatitis, hemolytic anemia and haemorrhagic diathesis, 2 patients with liver cirrhosis, haemorrhagic diathesis, Kayser-Fleisher ring, neuropsychiatric syndrome, 2 asymptomatic patients without haemorrhagic diathesis. The prothrombin index and the factors of prothrombin stem (II, V, VII, X) were lower than in other kinds of cirrhosis. After treatment with d-penicillamine the clothing factors returned near to the norm, similar as the biochemical and immunological results.

Adolescent↗
PubMed1999

Meningococcal disease in a large urban population (Barcelona, 1987-1992): predictors of dismal prognosis. Barcelona Meningococcal Disease Surveillance Group.

CONTEXT: Studies on meningococcal disease in large urban communities have rarely been performed and are usually based on passive epidemiologic surveillance. Active surveillance may provide new insights. OBJECTIVES: To determine epidemiologic, clinical, and bacteriological characteristics and predictors of dismal prognosis (death and sequelae) in meningococcal disease. DESIGN: Prospective, population-based study. SETTING: All the acute care hospitals (n = 24) in Barcelona, Spain. PATIENT: The 643 patients whose conditions were diagnosed from 1987 through 1992 were detected by 2 active surveillance methods. OUTCOME MEASURES: Incidence and notification to Public Health Service. Clinical and bacteriological features were determined. Dismal prognosis predictors were determined by logistic regression. RESULTS: Average annual incidence was 6.41 per 100,000 inhabitants, with no clear trend of change (P = .08). Sensitivity of the Public Health Service surveillance system was 69.1%. Children younger than 10 years from the inner city were at higher risk than those from the highest income district (relative risk, 3.00; 95% confidence interval [CI], 1.84-5.06). Increasing annual incidence of serogroup C (0.82-1.29/100,000; P = .008) and decreasing incidence of serogroup B (5.11-2.82/100,000; P = .004) was noted. Average annual mortality was 0.40 per 100,000 inhabitants, while the annual average potential years of life lost was 18 per 100,000 inhabitants. Overall case-fatality rate was 6.4%. Independent predictors of death were hemorrhagic diathesis (odds ratio [OR], 63; 95% CI, 21-194), focal neurologic signs (OR, 10; 95% CI, 3-30), and age 60 years or older (OR, 6; 95% CI, 2-17), whereas preadmission antibiotic therapy was associated with favorable outcome (OR, 0.07; 95% CI, 0.02-0.3). Four percent of survivors presented with sequelae. Independent predictors of sequelae were hemorrhagic diathesis (OR, 21; 95% CI, 3-131), focal neurologic signs (OR, 16; 95% CI, 5-53), age 60 years or older (OR, 7; 95% CI, 2-26), and age between 15 and 59 years (OR, 5; 95% CI, 2-14), whereas preadmission antibiotic therapy had a protective effect (OR, 0.2; 95% CI, 0.04-0.5). CONCLUSIONS: Active epidemiologic surveillance significantly improved detection of cases and allowed us to observe that meningococcal disease still causes much morbidity and mortality, especially among children living in the inner city. Hemorrhagic diathesis, focal neurologic signs, and age were independent predictors of dismal prognosis, whereas preadmission antibiotic therapy had a protective effect.

Adolescent↗
PubMed1997

Prognostic factors in meningococcal disease. Development of a bedside predictive model and scoring system. Barcelona Meningococcal Disease Surveillance Group.

CONTEXT: Meningococcal disease is associated with significant morbidity and mortality. Development of a prognostic model based on clinical findings may be useful for identification and management of patients with meningococcal infection. OBJECTIVES: To construct and validate a bedside model and scoring system for prognosis in meningococcal disease. DESIGN: Prospective, population-based study. SETTING: Twenty-four hospitals in the metropolitan area of Barcelona, Spain. PATIENTS: A total of 907 patients with microbiologically proven meningococcal disease. Patients diagnosed with meningococcal disease from 1987 through 1990 were used to develop the prognostic model, and those diagnosed in 1991 and 1992 were used to validate it. OUTCOME MEASURES: Clinical independent prognostic factors for mortality in meningococcal disease. The association between outcome and independent prognostic factors was determined by logistic regression analysis. A scoring system was constructed and tested using receiver operating characteristic curves. RESULTS: Among 624 patients in the derivation set, 287 (46%) were male, the mean age was 12.4 years, and 34 patients (5.4%) died. Among 283 patients in the validation set, 124 (43.8%) were male, the mean age was 12.7 years, and 17 patients (6.0%) died. In multivariate analysis, independent predictors of death were hemorrhagic diathesis (odds ratio [OR], 101; 95% confidence interval [CI], 30-333), focal neurologic signs (OR, 25; 95% CI, 7-83), and age 60 years or older (OR, 10; 95% CI, 3-34), whereas receipt of adequate antibiotic therapy prior to admission was associated with reduced likelihood of death (OR, 0.09; 95% CI, 0.02-0.4). Hemorrhagic diathesis was scored with 2 points, presence of focal neurologic signs with 1 point, age of 60 years or older with 1 point, and preadmission antibiotic therapy was scored as -1. The clinical scores of -1, 0, 1, 2, and 3 or more points were associated with a probability of death of 0%, 2.3%, 27.3%, 73.3%, and 100%, respectively. CONCLUSIONS: Hemorrhagic diathesis, focal neurologic signs, and age of 60 years or older were independent predictors of death in meningococcal disease, whereas receipt of adequate antibiotic therapy was associated with a more favorable prognosis. The scoring system presented is simple, is based on findings readily available at the bedside, and may be useful to help guide aggressive therapy.

Adolescent↗
PubMed1998

Acquired Glanzmann's thrombasthenia associated with Hodgkin's lymphoma: a case report and review of the literature.

BACKGROUND: Acquired Glanzmann's thrombasthenia is a rare hemorrhagic diathesis resulting from impaired adhesive function of the platelet receptor GPIIb/IIIa (alpha(IIb)beta3). Typically, this disorder develops during adulthood, with patients manifesting fluctuating clinical and laboratory findings. To date, the underlying defect of most if not all cases of acquired Glanzmann's thrombasthenia results from an autoantibody or plasma protein inhibitor directed toward a demonstrably normal GPIIb/IIIa glycoprotein. METHODS: In this report, a patient with a history of treated Hodgkin's lymphoma presented with a severe hemorrhagic diathesis characterized by mild thrombocytopenia, a prolonged bleeding time, and defective platelet aggregation. RESULTS: Examination of the patient's platelet GPIIb/IIIa by Western blot analysis revealed no abnormality. Mixing studies demonstrated a non-immunoglobulin G plasma inhibitory factor, whereas flow cytometry analysis revealed elevated platelet-associated immunoglobulin (Ig) M. After an emergency colectomy for severe hemorrhage, the patient's qualitative and quantitative platelet parameters significantly improved. Pathology of the resected colonic segment demonstrated atypical lymphoid hyperplastic lesions. CONCLUSIONS: To the authors' knowledge, this is the first reported case of acquired Glanzmann's thrombasthenia associated with a putative IgM autoantibody. Furthermore, this report verifies the association of acquired thrombasthenia with lymphoproliferative disease. Although rare, awareness of this hemorrhagic diathesis as a possible sequelae of active or treated lymphoid disorders should encourage clinical vigilance of these patients.

Antibodies, Neoplasm↗
PubMed1993

Biological individuality and disease. From Garrod's Chemical Individuality to HLA associated diseases.

The concept of "predisposition" in medicine is ancient, and the term "diathesis" was used to express it since the days of Hippocrates and, especially, of Galen. The concept of diathesis was enormously popular throughout the nineteenth century, despite the vagueness of its actual meaning. It was clarified only in the early years of the twentieth century (1902), when it was however losing its clinical relevance, by a replacement of the concept of chemical individuality by A.E. Garrod, followed thirty years later by the concept of inborn factors in disease (1931). "Molecular" knowledge of the biological individuality of human beings, highlighted particularly by the discovery of the MHC (Major Histocompatibility Complex) during the last 30-35 years, and substantially HLA (Human Leukocyte Antigens), has offered a new and better understanding of the relationship between the self and the not-self, as well as of various diseases, especially if they are favored by some immune dyshomeostatis. Extensive knowledge of transplants--their "immune" fate of take or rejection, possibly of GVHD--have allowed mankind to consider each human being as a biological Ego, unique in his antigenic-molecular structure. But most of all, the demonstration of the fact that certain HLA antigens can be significantly associated with a greater predisposition, on the part of individuals bearing these antigens, toward contracting certain diseases, reconsiders in precise molecular terms the concept of "predisposition" and therefore, perhaps, in a new light, even the concept of "diathesis", providing an actual logical basis for it.

Disease Susceptibility↗
PubMedDate not supplied

Changes in blood chemistry, hematology, and histology caused by a selenium/vitamin E deficiency and recovery in chicks.

Exudative diathesis, a condition caused by a selenium (Se)/vitamin E deficiency, was studied in chicks. Trios of chicks that showed clinical signs of exudative diathesis were matched for severity. One was injected subcutaneously with 0.5 mL distilled water, and the other two received 15 microg of Se in 0.5 mL distilled water. A chick fed a diet with supplemental Se also received 0.5 mL distilled water. Blood was collected from three chicks 2 d after injection, and from the other chick, 6 d after injection. After blood was collected, pectoral muscle and bone marrow were collected. Deficient chicks showed varying degrees of necrosis in pectoral muscle, whereas recovering chicks had extensive fibrosis in pectoral muscle. An analysis of blood showed differences in CO2, glucose, Se, glutathione peroxidase, alanine aminotransferase, aspartate aminotransferase, and creatine kinase. Heterophils and monocytes were increased in deficient chicks; lymphocytes, basophils, and hemoglobin decreased. After 6 d of recovery, all of the changes noted above were correcting toward normal. Eosinophils, in contrast, were unaffected by a deficiency, but increased in recovering chicks. It is hypothesized that cytokines associated with the inflammatory response accentuate the clinical signs of exudative diathesis.

Animals↗
PubMed2006

Thrombosis and bleeding in polycythemia vera and essential thrombocythemia: pathogenetic mechanisms and prevention.

Polycythemia vera and essential thrombocythemia are chronic myeloproliferative disorders, the benign clinical course of which can be complicated by both thrombotic and hemorrhagic diatheses. Thrombotic diathesis is characterized by microcirculatory disturbances and by an increased risk of arterial and venous thromboses. Thrombotic accidents often manifest at diagnosis or in the preclinical phase of the disease so that the search for a latent myeloproliferative disorder has become widely recommended in screenings for acquired thrombophilia, particularly when venous thromboses manifest at an unusual site. Hemorrhagic diathesis is more rare, less ominous and mostly affects patients with a very high platelet count. In these subjects, an altered degradation and function of von Willebrand factor can cause minor mucocutaneous hemorrhages, which are sometimes a prelude to major gastrointestinal bleedings. The bleeding tendency can be effectively treated by cytoreduction. Pathogenesis and treatment of thrombotic diathesis are still controversial. The nature of disease-related hemostatic abnormalities and the role of common risk factors are far from being elucidated. In polycythemic subjects, treatment of blood hyperviscosity is essential and low-dose aspirin, which has an established antithrombotic efficacy, should always be used in the absence of contraindications. These are mostly constituted by conditions of increased bleeding risk, which, in particular, have to be evaluated when considering aspirin use in patients with essential thrombocythemia. Future clinical research should primarily aim to assess the risk/benefit ratio of aspirin use in this disease, to better characterize the determinants of vascular risk and to reduce the high incidence of leukemias in patients with these diseases. This might require either the availability of safer cytoreductive agents or, alternatively, the use of more aggressive antiplatelet regimens in patients at high thrombotic risk.

Hemorrhage↗
PubMed2006

Functional magnetic resonance imaging study of cognitive control in the healthy relatives of schizophrenia patients.

BACKGROUND: The predisposition, or diathesis, to schizophrenia is highly heritable. The manner in which this genetic diathesis is manifest in the central nervous system is largely unknown, although healthy relatives of schizophrenia patients show executive processing deficits associated with prefrontal cortical impairments. METHODS: The current study evaluated brain activity in 21 healthy relatives of schizophrenia patients and 20 demographically similar control subjects during correct trials on a stimulus-response incompatibility task. During the first part of each trial, participants represented and maintained the instruction for that trial; during the second part, participants used the instruction either to make an automatic response or to overcome this prepotent response. RESULTS: Behaviorally, relatives were slower when overcoming the prepotent response. Analyses focused on the first part of the trial indicated that both groups showed activity in middle frontal (Brodmann areas 46 and 9) and anterior cingulate (Brodmann area 32) gyri. However, control subjects showed significantly greater activity in dorsal prefrontal cortex (Brodmann areas 9, 8, and 6) when preparing to overcome the prepotent response, whereas patients' relatives showed prefrontal activity later, when making the response. CONCLUSIONS: Using an event-related design showed distinct prefrontal brain abnormalities associated with the genetic diathesis to schizophrenia.

Adult↗
PubMed2005

Successful bilateral lung transplantation for pulmonary fibrosis associated with the Hermansky-Pudlak syndrome.

Hermansky-Pudlak syndrome (HPS) is a genetic disorder characterized by oculocutaneous albinism, a bleeding diathesis, and in a subset of patients, pulmonary fibrosis. Lung transplantation, the only curative therapy for pulmonary fibrosis, has not been previously reported as a successful treatment strategy for patients with HPS because the bleeding diathesis was thought to contraindicate major thoracic surgery. We successfully performed bilateral sequential lung transplantation in a patient with pulmonary fibrosis and HPS after transfusion of 6 units of platelets. Lung transplantation is a viable therapeutic option in patients with pulmonary fibrosis and only a mild bleeding diathesis associated with HPS.

Adult↗
PubMed2005

Stability and change of emotional functioning in late life: modelling of vulnerability profiles.

BACKGROUND AND AIMS: The present study investigated stability and change in emotional well-being in a prospective study of a large sample of community-dwelling older adults (> or = 55 years). Emotional functioning was conceptualized according to the tripartite model distinguishing three aspects: general negative affect (NA), depression, and anxiety. The study tested models for the decline of mental health in late life based on the diathesis-stress model. In previous studies, support has been found for the diathesis-stress model (for an overview, see [Goldberg, D.P., Huxley, P., 1992. Common mental disorders: a biosocial model. Routledge, London; Zuckerman, M., 1999. Vulnerability to psychopathology. American Psychological Association, Washington, DC.]). The predictive ability of vulnerability factors (the personality characteristics mastery and neuroticism) and stressful life events and their interaction was tested for an increase in general negative affect, decreased positive affect (PA), and increased anxiety. More specifically, we tested the hypothesis that loss leads to decreased positive affect in subjects with low mastery, whereas threat leads to anxiety in subjects with high neuroticism. METHODS: Data from the Longitudinal Aging Study Amsterdam (LASA) were used. LASA is a longitudinal study in a large representative sample of adults aged 55 to 85 (N=1837). Self-report data on depression, anxiety, and negative affect were collected from adults over a 6-year period in three waves. The data were analyzed using multilevel analysis. RESULTS: The findings revealed an association between low mastery, high neuroticism, and an increase in negative affect, lack of positive affect, and anxiety. Furthermore, high mastery protected against the negative impact of loss events, but neuroticism did not augment the negative impact of threat events on emotional health. CONCLUSION: Partial support was found for a diathesis-stress model of change in emotional functioning in late life. Furthermore, support was found for distinguishing between symptoms of negative affect, depression, and anxiety.

Affect↗
PubMed2004

An objective method to evaluate the risk of recurrence and extension of Dupuytren's disease.

This study was undertaken to assess the influence of the factors related to Dupuytren's diathesis on the rates of recurrence and extension of Dupuytren's disease after surgery. The records of 65 patients who underwent surgery for Dupuytren's disease were retrospectively studied and the presence of factors related to diathesis were recorded. The sensitivity and specificity of each factor for predicting recurrence and extension were calculated. Odds ratios and 95% confidence intervals were also calculated and a discriminant analysis was performed to explore correlations between recurrence and extension and the significant variables. Our results confirmed the prognostic value of diathesis. The results have been used to develop a new scoring system for evaluating the risk of recurrence and extension.

Adult↗
PubMed2007

The effect of supervised exercise and cilostazol on coagulation and fibrinolysis in intermittent claudication: a randomized controlled trial.

BACKGROUND: The prothrombotic, hypofibrinolytic state that develops in patients with intermittent claudication (IC) upon walking due to ischemia-reperfusion injury (IRI) of the leg muscles may contribute to the high incidence of life- and limb-threatening thrombotic events observed in this patient group. Treatments, such as angioplasty, that obtund the IRI also ameliorate the procoagulant diathesis. The effect on this diathesis of supervised exercise and cilostazol, both of which provide symptomatic benefit in IC, but without significantly obtunding IRI, is unknown. METHODS: Thirty-four patients (27 men and 7 women; median age, 67 years; range, 63-72 years) were randomized to receive best medical therapy (BMT) plus supervised exercise (n = 9), BMT plus cilostazol (n = 9), BMT plus supervised exercise plus cilostazol (n = 7), or BMT alone (n = 9) in a 2 x 2 factorial design. Thrombin-antithrombin complex and prothrombin fragments 1 and 2, both markers of thrombin generation; plasminogen activator inhibitor antigen and tissue plasminogen activator antigen, both markers of fibrinolysis; ankle-brachial pressure index (ABPI); and initial and absolute claudication distance (ACD) were measured at baseline and then 3 and 6 months after randomization. RESULTS: At 6 months, when compared with receiving BMT only, supervised exercise and cilostazol resulted in improvements in ABPI of 18% and 13% and in ACD of 40% and 64%, respectively. The effects on ABPI and ACD of combining supervised exercise and cilostazol were additive. Supervised exercise, cilostazol, and supervised exercise combined with cilostazol had no significant effect on any of the four hemostatic markers. CONCLUSIONS: Treatment of IC by supervised exercise or cilostazol results in significant improvements in ABPI and ACD but has no demonstrable effect on the prothrombotic diathesis. This suggests that supervised exercise and cilostazol, unlike angioplasty, are unlikely to have a long-term beneficial effect on the thrombotic risks faced by these patients.

Aged↗
PubMed1998

Thrombocytopenia, giant platelets, and leukocyte inclusion bodies (May-Hegglin anomaly): clinical and laboratory findings.

PURPOSE: May-Hegglin anomaly is a rare hereditary condition characterized by the triad of thrombocytopenia, giant platelets, and inclusion bodies in leukocytes. Clinical features and the pathogenesis of bleeding in this disease are poorly defined. PATIENTS AND METHODS: From 1988 to 1996 we studied 15 new May-Hegglin anomaly patients from 7 unrelated Italian families. In addition to clinical examination and routine laboratory testing, we measured bleeding time, platelet aggregation and release reaction, and platelet staining for tubulin, and performed ultrastructural study of polymorphonuclear leukocytes. RESULTS: Although the mean age of our patients was 33 years, May-Hegglin anomaly had not been previously recognized in any of them. Bleeding diatheses ranged from severe to absent, and platelet count from 26 to 178 x 10(9)/L. No correlation was found between bleeding tendency and platelet count. Previous therapy with corticosteroids, high-dose immunoglobulins, and splenectomy had no effect on platelet count or bleeding diathesis. Desmopressin infusion greatly shortened the bleeding time in the most severely affected patient. The in vitro function of platelets was normal except for the absence of shape change in all subjects and defective response to epinephrine in 8 of 15 patients. Platelet tubulin was distributed unevenly instead of being organized in a circumferential band at the cell periphery. CONCLUSION: The diagnosis of May-Hegglin is easily missed, and its frequency is probably underestimated. A qualitative defect of platelets may be responsible for mild bleeding diathesis even in the absence of thrombocytopenia, while severe bleeding results from both qualitative and quantitative platelet defects. May-Hegglin anomaly should be suspected whenever a patient has a low platelet count or a bleeding diathesis of unknown origin.

Adolescent↗