Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the appearance of a factor V inhibitor in an 82-year-old female patient following abdominal surgery. This anomaly was totally corrected following 9-day treatment with i.v. immunoglobulins (0.4 g/kg/day), which allowed a needed further surgical intervention to be performed without hemorrhagic diathesis. The activity of this inhibitor was partly reversed by the in vitro addition of immunoglobulins, suggesting a concentration-effect relationship. This finding supports the hypothesis that anti-idiotypic antibodies could play a role in the therapeutic effect of immunoglobulins in such situations.
Schweiz Med Wochenschr 1992 Dec 26
PMID:[Acquired factor V inhibitor treated with intravenous immunoglobulins]. 147 62

A ruptured cerebral aneurysm in a 50-year-old female with idiopathic thrombocytopenic purpura was successfully clipped after preoperative high-dose gamma-globulin therapy to control the hemorrhagic diathesis. High-dose gamma-globulin therapy with or without steroid and/or platelet transfusion is recommended for such cases if the blood pressure can be controlled and the neurological condition permits delayed surgery.
Neurol Med Chir (Tokyo) 1991 Dec
PMID:Ruptured cerebral aneurysm successfully clipped in idiopathic thrombocytopenic purpura after high-dose gamma-globulin therapy--case report. 172 30

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.
Am J Hematol 1987 Dec
PMID:Reliability of absent platelet dense bodies as a diagnostic criterion for Hermansky-Pudlak syndrome. 312 May 78

Three of 13 patients with intracranial meningiomas showed the pre- and postoperative elevation of tissue-type plasminogen activator (t-PA) related fibrinolytic activity in euglobulin fractions (EFA). During operation, two of these three patients showed a significant elevation of the level of fibrin(ogen) degradation products and oozing in the operating field. However, oozing was not observed in the third patient who had been given tranexamic acid preoperatively. Fibrin autography revealed that a broad lytic band of mol wt 50-60 kDa, probably free t-PA, appeared in the plasma obtained from two of the three patients after operation when EFA elevated significantly. In all patients studied, the t-PA antigen levels were normal preoperatively but increased both during and after operation, and correlated mainly with the intensities of a lytic band of mol wt 110 kDa, probably t-PA complexed with its major inhibitor (PAI-1). These results suggest that the excessive fibrinolysis can induce the local hemorrhagic diathesis during operation and may be related to t-PA function in plasma.
Thromb Haemost 1988 Dec 22
PMID:Tissue-type plasminogen activator in patients with intracranial meningiomas. 314 49

We present the case of a woman affected by ovarian cancer metastatic to multiple lymph node and the CNS. She was affected by hemorrhagic diathesis with microangiopathic alterations, whereas coagulopathy developed only after some days in coincidence with disease worsening. Our patient is probably one of those in which cancer leads to microangiopathy and coagulopathy by means of a tissue factor-like activity, a common event in mucin secretory tumors. Fibrinolytic activity was also increased in our patient as in others of the same type. The main aspect of this case report is metastasis to the CNS and to other multiple sites, which is quite uncommon in such cancers. We retain that tumor procoagulant activity could have played a role in this phenomenon.
Tumori 1988 Dec 31
PMID:CNS metastasis in ovarian cancer with microangiopathic hemolytic anemia associated with diffuse intravascular coagulation. 323 17

Acute fatty liver of pregnancy (AFLP) is a rare, but often fatal disorder in the third trimester of pregnancy. We report on a patient with AFLP complicated by acute hemorrhagic diathesis, who survived after early diagnosis and adequate treatment. On electronmicroscopy, the characteristic microvesicular fatty deposits in the centrolobular hepatocytes could be demonstrated.
Z Gastroenterol 1986 Dec
PMID:[Acute fatty liver of pregnancy. A case report of the differential diagnosis of pregnancy-associated liver diseases]. 354 7

Amongst 13 subjects with albinism observed in a village in the Valais, 7 present the clinical and laboratory findings of the Hermansky-Pudlak syndrome. Because of the close family links and the numerous consanguine marriages present in the pedigree, it is probable that the 5 subjects who could not be examined also suffer from the same syndrome. It has an autosomal recessive mode of inheritance. In this isolate the gene and heterozygote frequencies are, respectively, 1:12 and 1:6. Clinically the hemorrhagic diathesis is moderate, the visual and cutaneous symptoms predominate. The platelet dysfunction results from a lack of storage of components (Ca++, nucleotides, amines) normally present in the dense granules. The lack of dense granules is demonstrated by electron and fluorescence microscopy. This structural anomaly is present in the megacaryocyte before individualization of the platelets. Giant melanosomes were found in skin melanocytes.
Helv Paediatr Acta 1983 Dec
PMID:[Hermansky-Pudlak syndrome in a Valais village]. 666

Acute promyelocytic leukemia (APL) is a rare subtype of acute myelogenous leukemia that is usually associated with a fatal hemorrhagic diathesis. All trans-retinoic acid (ATRA) is an active metabolite of vitamin A that differentiates the malignant cell clone, corrects the coagulopathy, and induces complete remission in the vast majority of patients with APL. Between June 1992 and September 1993, 8 patients with APL (4 previously untreated, 3 in first relapse and 1 in second relapse) received ATRA. Complete remission was achieved in 7 patients; in 5 with ATRA alone and in 2 with ATRA followed by cytotoxic chemotherapy due to the development of asymptomatic hyperleukocytosis. The earliest signs of response were the correction of the coagulopathy and an increase in the white blood cell count. Sequential morphological and immunophenotypical analyses of the bone marrow revealed differentiation of the malignant cell clone, in the absence of bone marrow hypoplasia. 4 of 5 patients treated only with ATRA until complete remission had late leukopenia. The most frequent adverse effects were dryness of skin and mucosae, hypertrigliceridemia and hypercholesterolemia, and a moderate increase in liver transaminases. An increase in the white blood cell count was common, and in two cases exceeded 35.0 x 10(9)/l. One of these patients developed multiple thrombosis of the extremities after cytotoxic chemotherapy. We frequently observed an increase in lactic dehydrogenase levels that was concomitant with the peak in the white blood cell count. The only patient on whom complete remission was not achieved was 60 years old, had chronic obstructive pulmonary disease, and died in the third week of therapy with a pulmonary distress syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
Acta Med Port 1994 Dec
PMID:[Treatment of acute promyelocytic leukemia with trans-retinoic acid. Experience of the Santa Maria Hospital, Medical School of Lisbon]. 771 19

This is a clinical study that attempts to determine the incidence and patterns of cutaneous lesions in 109 pediatric patients with leukemia. Non-specific lesions, namely, adverse reactions to chemotherapy, complications of immunosuppression, and hemorrhagic diathesis were seen in 88.7% of those patients with acute lymphocytic leukemia and in 88.9% of patients with acute non-lymphocytic leukemia. Leukemia cutis was seen in three patients with acute non-lymphocytic leukemia, and in one patient with congenital leukemia. It seems that although skin complications are a frequent event in the course of childhood leukemia, leukemic infiltration of the skin a is rare event.
P R Health Sci J 1994 Dec
PMID:The spectrum of cutaneous lesions in pediatric patients with leukemia. 789 42

The lupus anticoagulant may be accompanied by an acquired factor II deficiency and bleeding. We report on a patient with a lupus anticoagulant and factor II (Fll) deficiency responsive to Danazol. Acquired hypoprothrombinemia (FII) with the lupus anticoagulant (LA) may be accompanied by a hemorrhagic diathesis. A 64-year-old male with discoid lupus erythematosis bled after an intestinal polypectomy. His FII level was 18%, and his FII antigen level was 20%. Danazol (D) (600 mg per day) administration was associated with a rise in FII activity and antigen to 50% within 10 days. The patient underwent abdominal surgery. We studied the effect(s) of D on the FII level and on other coagulation factors in this patient. The patient's plasma FII antigen had a single precipitin arc compared to the two peaks of normal plasma on counterimmunoelectrophoresis with Ca++. The samples pre- and during D therapy had the same positively charged arc as normal samples, although they were quantitatively different. Neuraminidase treatment demonstrated a decrease in the positively charged migration of normal and the patient's FII antigen. Affinity chromatography of normal and patient plasma on a Sepharose protein A column revealed FII antigen present in the patient's bound fraction. The relative percentages of bound FII before and during D treatment were similar. During D therapy, levels of FIX and X rose 50-100%, and protein C rose 20-25%, while free protein S did not change. D is an effective therapy for acquired FII deficiency associated with LA. D does not affect the binding of Ig to FII, but D raises FII levels by increasing synthesis of the FII protein.
Am J Hematol 1996 Dec
PMID:Acquired hypoprothrombinemia: effects of danazol treatment. 894 70


<< Previous 1 2 3 4 Next >>