Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Haemophilia is uncommon in females and little is known about the clinical manifestations and postpartum management of women with this disorder. Clinical characteristics of postpartum bleeding were evaluated in women with factor IX deficiency (FIX:C < 0.20 U mL(-1)), including two with haemophilia B and three carriers of haemophilia B, undergoing labour and delivery. Data were collected prospectively during routine outpatient comprehensive haemophilia care at the haemophilia Center of Western Pennsylvania and during inpatient management. Four of five women experienced postpartum bleeding, during six of 16 deliveries: the median haemoglobin was 10.7 g% and two required blood transfusion. Postpartum bleeding was significantly more common among those receiving fewer than 4 days of FIX replacement: six of 13 (46.1%) receiving fewer three or fewer days bled vs. none of three (0%) receiving six or more days treatment [P < 01 (Wilcoxon)]. Postpartum bleeding was not related to the route of delivery (P = 0.525), vaginal vs. Caesarean, nor the FIX level (P = 0.371; FIX > 0.05 U mL(-1) vs. < or =0.05 U mL(-1)). Compared with females with von Willebrand disease or FXI deficiency, females with FIX deficiency were more likely to experience postpartum bleeding (P = 0.008) and anaemia (P = 0.045); and they were less likely to experience menorrhagia (P = 0.065), but the latter did not reach significance. Postpartum bleeding is common in women with haemophilia B or carriers of haemophilia B, and treatment with factor replacement for at least 4 days of postpartum may prevent bleeding following delivery in such women.
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PMID:Clinical manifestations and management of labor and delivery in women with factor IX deficiency. 1535 75

The reported prevalence of von Willebrand's disease (vWD) is increased in women with menorrhagia, with current estimates ranging from 5% to 20%. The consistent results of multiple studies suggest testing should be included in the evaluation of patients with menorrhagia, especially in unexplained cases and prior to surgical intervention. Although a cyclic variation in von Willebrand's factor levels has not been confirmed, several studies suggest lower levels during menses and the early follicular phase. Menorrhagia is one of the most common bleeding manifestations of von Willebrand's disease, reported by 60-95% of women afflicted with this bleeding disorder. Menorrhagia is typically severe, often resulting in anemia and interfering with quality of life. Despite the frequency of menorrhagia, there is no consensus on optimal management. Although oral contraceptives are frequently prescribed, there are no studies confirming their efficacy using objective measures of response. Desmopressin was associated with an 80-92% response rate in several uncontrolled studies relying on patient assessment of efficacy. However, a small, randomized trial found no significant reduction in menstrual blood flow compared with placebo. There are anecdotal reports of the successful use of antifibrinolytic agents alone and in combination with other therapies. There are no studies comparing the relative efficacy and safety of the available medical therapies for von Willebrand's disease associated menorrhagia. Until these studies are completed, treatment should be individualized based on von Willebrand's disease subtype, patient age, contraceptive needs, and personal preference.
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PMID:von Willebrand's disease and menorrhagia: prevalence, diagnosis, and management. 1598 Dec 34

Prophylaxis with von Willebrand factor (VWF)-containing concentrates is considered to be a potential approach for patients with von Willebrand disease (VWD) and severe bleeding tendency. We report the case of a 57-year-old man with type 3 VWD and a history of recurrent melaena. Bleeding frequency and severity had progressively increased and the patient showed chronic anaemia and persistent haemoglobin in the stools. Endoscopic examinations revealed multiple vascular mucosal abnormalities (MVA) of the stomach and large bowel. Photocoagulation of some actively bleeding lesions and octreotide did not significantly affect his clinical conditions: six red cell transfusions and >400 000 IU of intermediate-purity factor VIII (FVIII) concentrate (Haemate P) on-demand were needed during 2002. Prophylaxis with Haemate P 40 IU kg(-1) (102 IU kg(-1) VWF:RCo) thrice weekly was associated with improvement of his mean haemoglobin levels, cessation of clear-cut melaena and red cell transfusions and reduction of total Haemate P requirements (-20% over 2003-04). Prophylaxis with Haemate P is still ongoing without any adverse event over a 30-month period. Clinical course and pharmacokinetic evaluations led to administer Haemate P each 72-96 h. Possible vascular complications were excluded by a careful clinical follow up, as the patient suffered from arterial hypertension and diabetes mellitus; thrombophilic abnormalities were previously excluded and no signs of abnormal coagulation activation or FVIII:C levels >150% were detected thereafter. Long-term prophylaxis with Haemate P has been shown to be safe, effective (also in terms of quality of life) and cost saving in this patient with severe gastrointestinal bleeding due to MVA and VWD.
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PMID:Long-term prophylaxis with intermediate-purity factor VIII concentrate (Haemate P) in a patient with type 3 von Willebrand disease and recurrent gastrointestinal bleeding. 1640 82

Watermelon stomach (WMS), or gastric antral vascular ectasia, is an uncommon but clinically important cause of chronic occult or overt gastrointestinal (GI) blood loss. Patients typically present with symptomatic anemia and hemoccult positive stools. Although the diagnosis is based primarily on the typical endoscopic appearance, the lesion may be overlooked on initial evaluation or interpreted as "gastritis." Gastric biopsy may be helpful in confirming the diagnosis by showing vascular ectasia, typically without inflammation. WMS is idiopathic but is often associated with autoimmune diseases or cirrhosis of the liver. The majority of patients with classic WMS are elderly and female. In contrast, there is no gender predominance with the diffuse pattern associated with cirrhosis. General supportive care includes transfusion of blood products as necessary to control symptomatic anemia and coagulopathy, iron replacement therapy by oral or parenteral routes, identification and treatment of iatrogenic (eg, warfarin) or hereditary (eg, von Willebrand's disease) coagulopathy, and avoidance of substances that might cause gastric mucosal damage and/or bleeding (eg, aspirin, NSAIDs, alcohol). The goals of therapy are to eliminate or decrease the need for blood transfusions, hospitalization, office visits, and endoscopic therapy sessions aimed at cessation of GI blood loss and resolution of symptomatic anemia. Multipolar electrocoagulation is our preferred technique for endoscopic ablation of WMS. A 10-Fr probe (3.2 mm in diameter) is used at a generator setting of 12 to 16 W. Pulse duration can be as short as 1 to 2 seconds if a pinpoint coagulation technique is used, or continuous if a paint-stroke technique is used to coagulate all vascular stripes in the classic WMS pattern or as many small lesions as possible in the diffuse type. Other techniques we currently use are argon plasma coagulation (APC) or heater probe. In the past, lasers (neodymium: yttrium-aluminum-garnet , KTP, or argon) were successfully used for such treatments. An initial treatment interval of 4 to 8 weeks should allow for interim healing of iatrogenic ulcers. Patients are routinely given standard doses of available proton-pump inhibitors (PPIs) to facilitate healing of iatrogenic ulcers and to prevent secondary bleeding. The treatment interval can be gradually lengthened as the long-term goals of obliteration of angiomata and resolution of anemia are reached. Side effects may include iatrogenic ulceration at the site of treatment, bleeding, and transient abdominal pain. Antral scarring (after APC or Nd:YAG laser) and hyperplastic polyps have also been reported after endoscopic treatment of classic WMS. Surgical antrectomy is very effective in the prevention of bleeding but has substantial (5% to 10%) mortality in older patients with comorbidity and is now reserved for endoscopic failures.
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PMID:Treatment of watermelon stomach. 1653 78

Aortic valve stenosis can be complicated by recurrent gastrointestinal bleeding, particularly that due to angiodysplasia, also called Heyde syndrome. Recently, acquired type 2A von Willebrand disease, which is characterized by the loss of the large multimer of von Willebrand factor by the shear stress of aortic valve stenosis, was reported to be associated with this hemorrhagic syndrome. A 78-year-old woman, with severe aortic stenosis, presented with advanced anemia due to recurrent gastrointestinal bleeding and was diagnosed Heyde syndrome. By perioperative supplementation of von Willebrand factor and factor VIII, aortic valve replacement was safely performed without gastrointestinal bleeding. After the operation, the multimer of von Willebrand factor was normalized and thereafter no gastrointestinal bleeding occurred. This case reports the successful aortic valve replacement for Heyde syndrome, with confirmed hematologic recovery.
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PMID:Successful aortic valve replacement for Heyde syndrome with confirmed hematologic recovery. 1718 82

Menorrhagia, or excessive menstrual bleeding, is a common clinical problem affecting reproductive-age women; however, the cause is undetermined in 50% of cases. Von Willebrand disease (VWD) or other bleeding disorders may be the underlying source of heavy bleeding. Women with menorrhagia and/or VWD are at increased risk for several conditions including anemia, bleeding during pregnancy, post-partum hemorrhage, and reduced quality of life (OOL). Proper diagnosis and management can decrease complications and unnecessary surgical interventions. The Division of Blood Disorders (DBD) at the Centers for Disease Control and Prevention (CDC) has implemented studies to ascertain physician awareness of bleeding disorders, establish prevalence in the U.S., and determine the best treatment options.
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PMID:Women with bleeding disorders. 1800 Nov 80

Iron deficiency anaemia (IDA) is a frequently encountered disease, which can be attributed to menorrhagia. Most female patients with von Willebrand disease (VWD) have menorrhagia. The aim of this study was to investigate the prevalence of VWD in women with both IDA and menorrhagia in Taiwan. From January to December 2005 and November 2006 to January 2007, 56 consecutive patients with both IDA and menorrhagia were enrolled in this study. Their median age was 41 years (range 18-53). IDA was diagnosed by anaemia plus either low ferritin or transferrin saturation. Menorrhagia was evaluated by patient's menses history. Both von Willebrand factor antigen (VWF:Ag) and ristocetin cofactor activity (VWF:RCo) were measured for each patient. Bleeding time (BT) and platelet function analyser (PFA)-100 assay were determined as ancillary tests. The VWD diagnosis was established if: (i) both VWF:Ag (<50%) and VWF:RCo (<50%) were low; (ii) either VWF:Ag or VWF:RCo was low plus prolonged BT or prolonged PFA closure times. VWF multimer analysis was performed for subtype confirmation of VWD. Nine of the 56 (16.1%) patients were identified to have VWD. VWD patients with menorrhagia might develop IDA at younger age (34.3 vs. 39.7, P = 0.09) and had more IDA recurrence (75% vs. 16%, P = 0.03) than those patients without VWD. Of the eight VWD patients with VWF multimer analyses, all were revealed to have type I VWD. Our study demonstrates that VWD was not uncommon in women with both IDA and menorrhagia in Taiwan.
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PMID:Prevalence of von Willebrand disease in women with iron deficiency anaemia and menorrhagia in Taiwan. 1849 2

Patients with severe forms of von Willebrand's disease (VWD) may have frequent haemarthroses, especially when factor VIII (FVIII) levels are below 10 U/dL, so that some of them develop target joints like patients with severe haemophilia A. Some patients have recurrent gastrointestinal bleeding, often without lesions in the gastrointestinal tract, and need treatment every day or every other day. Finally, there are children who have epistaxis frequently and severely enough to cause anaemia. In these frequent and severe bleeders, the optimal therapy may be secondary long-term prophylaxis with von Willebrand factor (VWF)/FVIII concentrates rather than on-demand treatment on the occasion of bleeding episodes. The largest experience on such prophylaxis in VWD has been in Sweden in 35 patients with severe forms of VWD. Long-term prophylaxis was also implemented in a cohort of Italian patients with VWD: prophylaxis was used in seven patients with types 3 (n = 1 ), 2A (n = 4), 2M (n = 1) and type 1 (n = 1) VWD because of recurrent gastrointestinal bleeds and in four patients with type 3 VWD because of joint bleeds. Prophylaxis prevented bleeding completely in eight patients and largely reduced hospitalisation for blood transfusions in the remaining three. The cost-effectiveness of these prophylaxis regimens versus on-demand therapy will now be investigated in one large international study
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PMID:Prophylaxis of bleeding episodes in patients with von Willebrand's disease. 1910 7

A 5-year-old male cynomolgus monkey (Macaca fascicularis) with a clinical history of bleeding tendency, severe anaemia, thrombocytopenia and elevated serum concentration of liver-related enzymes was examined post mortem. Ecchymotic haemorrhages were present on the left eyelid and forehead. The liver, kidney and spleen were markedly enlarged and the kidneys had capsular petechiae. Microscopically, numerous atypical cells resembling myeloid cells were observed in the bone marrow, and myelofibrosis was present. Atypical cells were also present in the blood vessels of the liver, kidney, spleen, lymph nodes, lung, heart, bladder, adrenal gland and brain. Some neoplastic cells had oval or pleomorphic macronuclei and others were multinucleated. Immunohistochemically, the majority of the neoplastic cells had granular cytoplasmic expression of the megakaryocyte-associated antigens Von Willebrand Factor and CD61-IIIa, but were negative for myeloperoxidase. A diagnosis of acute megakaryocytic leukaemia (AMKL)-like disease was made. This would appear to be the first report of AMKL-like disease in non-human primates. This monkey was infected with simian retrovirus type D and it is possible that this viral infection was associated with the development of neoplasia.
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PMID:Acute megakaryocytic leukaemia (AMKL)-like disease in a cynomolgus monkey (Macaca fascicularis). 1915 98

Inherited bleeding disorders are potentially causes of menorrhagia and must be investigated if no specific cause is identified. The reported prevalence of inherited bleeding disorders is high in women with menorrhagia compared to the general population. The most frequent disorders reported are von Willebrand's disease and Factor XI deficiency. Menorrhagia is, also, a frequent finding in women with congenital bleeding disorders. Morever, menorrhagia represents the major cause of iron-deficiency anemia among women of reproductive age. Primary evaluation for an underlying disorder of hemostasis in a woman wih menorrhagia is a focused history for familiy and personal history of bleeding symptoms and a complete blood cell count. This will rule out thrombocytopenic bleeding and also assesses for the degree, if any, of anemia. Those women with a positive screen and normal platelet count should be evaluated with laboratory investigation including prothrombin time, activated partial thromboplastin time, factor VIII, VWF ristocetin cofactor and antigen. If initial hemostasis testing above is normal, then further hemostasis testing can be considered, especially in terms of platelet aggregation, in a multidisciplinary clinic.
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PMID:[Appropriate laboratory investigation in women with menorrhagia]. 1926 9


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