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Bleeding Diathesis

Bleeding Disorder

Bleeding diathesis is a term describing a propensity toward bleeding or hemorrhage, which can be a constitutive feature of numerous disorders of vascular, genetic, or autoimmune origin. The diagnosis rests on identifying the underlying cause through a detailed patient history and laboratory workup.


Presentation

The clinical presentation of bleeding is diverse, and it is imperative to recognize different forms of bleeding that may provide vital information about the underlying cause. Cutaneous (petechiae, epistaxis, purpuric spots, ecchymosis) or mucosal bleeding (for eg. gingival or conjunctival bleeding) rises suspicion toward platelet disorders (such as thrombotic thrombocytopenic purpura (TTP), splenic sequestration or drug-induced thrombocytopenia) and vascular abnormalities [1] [2]. Muscular, parenchymal, intracavitary (hemoperitoneum, hemothorax), or orificial bleeding can point to disorders of impaired coagulation, including hemophilia A and B, vitamin K deficiency, celiac disease, von Willebrand disease (vWD) and many other [1] [2] [3]. In fact, a tendency towards bleeding can be the first manifestation of patients with celiac disease (CD) [4]. In addition to the type of bleeding, signs such as excessive hemorrhage from cuts, easy bruising or intracranial bleeding may suggest some form of coagulopathy as well (vitamin K deficiency or any of the inherited genetic disorders) [3] [5]. Menorrhagia, especially when present in younger women, is a diagnostic hallmark of bleeding disorders in this population. The onset of bleeding is also an important clue, as bleeding diathesis may last throughout the entire childhood or just for a few days, depending on the underlying cause. Hepatosplenomegaly, tachycardia, skin or conjunctival pallor, and anemia presenting with a heart murmur are other notable symptoms. In very rare cases, eczema and recurrent infections accompanied by frequent bleeding episodes are highly specific for Wiskott-Aldrich syndrome, an X-linked genetic disease [2].

Down Syndrome
  • The first case known to us with Down's syndrome with transient abnormal myelopoiesis and diffuse hepatic necrosis is reported. The infant had prominent bleeding diathesis and hepatosplenomegaly.[ncbi.nlm.nih.gov]
Noncompliance
  • Celiac crisis usually has some underlying precipitating factor and is more common in patient with noncompliance with gluten free diet.[ncbi.nlm.nih.gov]
Easy Bruising
  • Nine of our patients indicated a relevant bleeding diathesis and complained of easy bruising, three reported spontaneous gum bleeding.[ncbi.nlm.nih.gov]
  • Conclusions: Easy bruising was the most common presenting symptom for which patients were referred to hematology.[bloodjournal.org]
  • In addition to the type of bleeding, signs such as excessive hemorrhage from cuts, easy bruising or intracranial bleeding may suggest some form of coagulopathy as well (vitamin K deficiency or any of the inherited genetic disorders).[symptoma.com]
  • Easy bruising, epistaxis, prolonged bleeding during menstruation, after tooth extraction, and after minor surgical procedures are often reported. Platelets lack the normal number of 'dense bodies'.[disorders.eyes.arizona.edu]
  • Patient information : See related handout on easy bruising and bleeding, written by the authors of this article. Author disclosure: No relevant financial affiliations. Bleeding and bruising are common symptoms in the primary care setting.[aafp.org]
Hematemesis
  • Signs: Abnormal Bleeding (multiple sites) Nasopharynx Epistaxis Bleeding Gums Gastrointestinal Hematemesis Melana Gynecologic Menorrhagia Postpartum Hemorrhage Musculoskeletal Muscle hematomas Hemarthrosis Skin Purpura Petechiae Trauma Excessive bleeding[fpnotebook.com]
  • Case Presentation An 18-year-old unmarried young Asian female came through the emergency department (ER) with a history of hematemesis for last twenty-four hours. The patient reported that this was the third episode of hematemesis in one day.[cureus.com]
  • A 40-year-old woman with epistaxis, hematemesis, and altered mental status. Martha E Laposata , Elizabeth M. Van Cott , Michael H. Lev The New England journal of medicine 2007 3 Excerpts Celiac disease. Peter H. R.[semanticscholar.org]
Conjunctival Pallor
  • Hepatosplenomegaly, tachycardia, skin or conjunctival pallor, and anemia presenting with a heart murmur are other notable symptoms.[symptoma.com]
Epistaxis
  • Correction of the bleeding diathesis to control the epistaxis required treatment of the tumour with chemotherapy.[ncbi.nlm.nih.gov]
  • Presenting symptoms in rare inherited platelet disorders were purpura, ecchymosis, epistaxis and bleeding gums, respectively.[ncbi.nlm.nih.gov]
  • Epistaxis is a common and difficult problem to manage in patients with bleeding disorders.[ncbi.nlm.nih.gov]
  • Here we come across a rare case of Sillence type IB Osteogenesis imperfecta with bleeding in the form of repeated epistaxis and petechial rash, which seem to have a collagenous link.[ncbi.nlm.nih.gov]
Radiculopathy
  • A 25-year-old woman presented with weakness, fatigue, neck and low-back pain, and progressive left S-1 radiculopathy.[ncbi.nlm.nih.gov]

Workup

A detailed patient history should be obtained in all patients with a bleeding diathesis, as it may reveal key findings such as prior use of aspirin or anticoagulant therapy, a prolonged duration of symptoms and the familial presence of bleeding disorders [2] [6]. The type and severity of bleeding noted during the physical examination are pivotal as well, but to determine the underlying cause, an extensive laboratory workup is necessary. A complete blood count (CBC) can detect anemia and more importantly thrombocytopenia (defined as platelet count <150,000/μL), which may occur either as a result of impaired function or insufficient production of platelets. Initial workup should also comprise prothrombin time (PT) and activated partial thromboplastin time (aPTT), to determine whether the extrinsic or intrinsic pathway is disturbed [3]. Prolonged PT suggests factor VII deficiency, while hemophilias A and B, Von Willebrand disease, and deficiency of factors XI and XII are the most likely causes of bleeding if aPTT is prolonged [2]. If both extrinsic and intrinsic pathways are defective, vitamin K deficiency, liver disease, use of anticoagulant therapy or deficiency of factors V, X, or II must be considered in the differential diagnosis [2]. Additional procedures include a peripheral blood smear, platelet function analysis (PFA), thrombin time, inflammatory parameters (erythrocyte sedimentation rate, C-reactive protein, fibrinogen and D-dimer), as well as the determination of individual coagulation factors [2] [3].

Hepatic Necrosis
  • The first case known to us with Down's syndrome with transient abnormal myelopoiesis and diffuse hepatic necrosis is reported. The infant had prominent bleeding diathesis and hepatosplenomegaly.[ncbi.nlm.nih.gov]

Treatment

  • Due to multiple comorbidities, it may be impossible to safely discontinue the anticoagulant treatment. Other modalities such as shock wave lithotripsy and PCNL are contraindicated in these patients, so ureteroscopic treatment may be the only option.[ncbi.nlm.nih.gov]
  • A practical guide to thromboprophylaxis in the treatment of urinary tract lithiasis has not yet been established.[ncbi.nlm.nih.gov]
  • Early diagnosis and prompt treatment of a bleeding diathesis is crucial for full neurological recovery.[ncbi.nlm.nih.gov]
  • Correction of the bleeding diathesis to control the epistaxis required treatment of the tumour with chemotherapy.[ncbi.nlm.nih.gov]
  • The treatment of the bleeding diathesis of renal failure remains unsatisfactory. 1-Deamino-8-D-arginine vasopressin (DDAVP) has recently been shown to shorten the prolonged bleeding time of renal failure and may as a result diminish the risk of clinical[ncbi.nlm.nih.gov]

Prognosis

  • Relatively favorable prognosis. With age, the tendency to bleeding is reduced. Mortal danger may be profuse bleeding and bleeding into vital organs.[eurodoctor.ucoz.com]
  • The prognosis is variable, with 25% of the patients dying of heart failure in the first year. 2 Indeed, cardiac surgery is frequently performed in these patients.[pediatrics.aappublications.org]

Etiology

  • Evaluation of all NS patients and their families for bleeding disorders should provide important information about the frequency and type of bleeding diatheses which occur and perhaps help to clarify the etiology and pathogenesis of NS.[ncbi.nlm.nih.gov]
  • KEYWORDS: Blood coagulation disorders/complications; blood coagulation factors/inhibitors; central nervous system diseases/etiology; hemophilia A; hemophilia B; hemorrhage; hemorrhage/etiology; humans intracranial hemorrhages/etiology; platelet transfusion[ncbi.nlm.nih.gov]
  • To investigate the etiology of the abnormal coagulopathy, further diagnostic studies including coagulation factor assays, platelet aggregation studies, replitase time, mixing studies using pooled normal plasma, and protamine were performed.[ncbi.nlm.nih.gov]
  • We considered PAI-1 deficiency to be the likely etiology of the congenital bleeding diatheses in these 4 cases.[ncbi.nlm.nih.gov]
  • Several etiological factors are responsible for bleeding diathesis. Of the factors, insufficient clotting happens to be the most common.[geekloveshealth.com]

Epidemiology

  • Analysis of the clinico-epidemiological data of patients with bleeding disorders is a useful tool for monitoring and improving their quality of care.[ncbi.nlm.nih.gov]
  • We aimed to assess the prevalence of various bleeding disorders including rare inherited coagulation and platelet disorders concerning their clinico-epidemiological, diagnostic data and bleeding manifestations severity.[journals.lww.com]
  • ., liver cirrhosis Other important types Von Willebrand disease Definition : : a disorder of primary hemostasis characterized by either a deficiency or disorder of vWF Epidemiology : most common congenital hemostatic disorder Clinical findings Mostly[amboss.com]
Sex distribution
Age distribution

Pathophysiology

  • Kwaan Springer Science & Business Media , ٢١‏/٠٤‏/٢٠٠٩ - 339 من الصفحات Coagulation in Cancer informs professionals working in the field of cancer about the pathophysiologic mechanisms of cancer-related thrombosis and bleeding.[books.google.com]
  • Such pathophysiology significantly stresses even an initially normal hemostatic mechanism. It is not surprising, then, that bleeding is a frequent complication encountered in the intensive care setting.[link.springer.com]
  • Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Bleeding problems may be associated with a wide variety of coagulation abnormalities or may be due to problems not associated[mayomedicallaboratories.com]
  • References: [1] [2] [3] [4] [5] [6] [7] [8] Pathophysiology Activation of hemostasis Primary hemostasis : hemostasis achieved via platelet adhesion and aggregation at the site of endothelial injury Initiation : endothelial injury results in transient[amboss.com]

Prevention

  • […] metopic craniosynostosis patient with guanine nucleotide-binding protein alpha stimulating (GNAS) mutation; perform a literature review regarding bleeding diathesis in craniosynostosis patients with GNAS mutations; and suggest guidelines to potentially prevent[ncbi.nlm.nih.gov]
  • Treatment with oral anti-fibrinolytic agents prevented further bleeding episodes. Decompensated cirrhosis eventually necessitated orthotopic liver transplantation.[ncbi.nlm.nih.gov]
  • Proper regulation of the fibrinolytic system is critical to the prevention of both thrombosis and hemorrhage.[ncbi.nlm.nih.gov]
  • We present a case of recurrent epistaxis in a patient with Bernard-Soulier syndrome (a platelet disorder) and describe a non-invasive but effective method of closing the nasal vestibule using a silastic obturator thus preventing the drying effects of[ncbi.nlm.nih.gov]
  • It provides assistance in recognizing the various bleeding and clotting disorders associated with cancer and includes current recommendations for the management of hemorrhage, and prevention and treatment of thrombosis in the patient with malignancy.[books.google.com]

References

Article

  1. Girolami A, Luzzatto G, Varvarikis C, Pellati D, Sartori R, Girolami B. Main clinical manifestations of a bleeding diathesis: an often disregarded aspect of medical and surgical history taking. Haemophilia. 2005;11(3):193-202.
  2. Bashawri LAM, Ahmed MA. The approach to a patient with a bleeding disorder: for the primary care physician. Journal of Family & Community Medicine. 2007;14(2):53-58.
  3. Tiplett DA. Coagulation and bleeding disorders: review and update. Clin Chem. 2000;46(8 Pt 2):1260-1269.
  4. Halfdanarson TR, Litzow MR, Murray JA. Hematologic manifestations of celiac disease. Blood. 2007;109(2):412-421.
  5. Kasatkar P, Shetty S, Ghosh K. Delayed vitamin K deficiency as a cause of bleeding: still a concern in the 21st century! Blood Coagul Fibrinolysis. 2010;21(6):608-610.
  6. Dizdar O, Onal IK, Ozakin E, et al. Research for bleeding tendency in patients presenting with significant epistaxis. Blood Coagul Fibrinolysis. 2007;18(1):41-43.

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Last updated: 2019-06-28 11:34