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Autoimmune Thrombocytopenia


Presentation

  • In the present report, we present a case with chronic HCV infection in which autoimmune thrombocytopenia developed at week 7 of PEG-IFN alpha 2b plus ribavirin therapy. The patient subsequently received ursodeoxycholic acid (UDCA) treatment.[ncbi.nlm.nih.gov]
Congestive Heart Failure
  • This manifestation was the cause of rapidly progressive congestive heart failure and, together with the unusual occurrence of autoimmune thrombocytopenia, led to difficult patient care with a significantly negative influence on the outcome.[ncbi.nlm.nih.gov]
  • Cardiopulmonary disease including: History of coronary artery disease, angina pectoris or congestive heart failure. LV ejection fraction less than 40 percent by 2D echocardiogram.[clinicaltrials.gov]
Death in Infancy
  • Children with severe combined immunodeficiency (SCID) have profoundly diminished humoral and cellular immunity resulting in death during infancy unless immune reconstitution occurs by bone marrow transplantation (BMT).[ncbi.nlm.nih.gov]
Easy Bruising
  • A decrease in platelets can result in easy bruising, bleeding gums, and internal bleeding. ITP may be acute and resolve in less than 6 months, or chronic and last longer than 6 months.[hopkinsmedicine.org]
  • The low platelet count causes easy bruising and bleeding, which may be seen as purple areas in the skin, mucous membranes, and outer linings of organs.[icd10data.com]
  • A decrease in platelets can result in easy bruising, bleeding gums and internal bleeding. "Idiopathic" means the cause is unknown. "Thrombocytopenia" means a decreased number of platelets in the blood.[chop.edu]
  • The clinical manifestations of bleeding were generally mild, such as easy bruising and, in the majority of patients the thrombocytopenia was detected incidentally.[scirp.org]
Petechiae
  • Patients with chronic autoimmune thrombocytopenia typically present with petechiae and mucosa bleeding. Symptoms may be present for months, or some patients may experience more acute manifestations.[asmscience.org]
  • CASE REPORT: A 70-year-old woman was admitted with rectal bleeding accompanied by widespread petechiae, bruising, tongue and buccal mucosa bleeding, and epistaxes and proved refractory to HLA- and HPA-matched PLTs.[ncbi.nlm.nih.gov]
  • References • Nachman RL, Rafii S: Platelets, petechiae, and preservation of the vascular wall, N Engl J Med 359:1261–1270, 2008. • Donato H, Picón A, Martinez M, et al.[slideshare.net]
  • These bruises may also appear as pinpoint-sized red or purple dots on the skin called petechiae. Petechiae often look like a rash. ITP can occur in both children and adults.[healthline.com]
  • When they do occur, they may include: Easy or excessive bruising Superficial bleeding into the skin that appears as pinpoint-sized reddish-purple spots (petechiae) that look like a rash, usually on the lower legs Bleeding from the gums or nose Blood in[mayoclinic.org]
Eczema
  • Veien NK (2010): Bullous pemphigoid masquerading as recurrent vesicular hand eczema. Acta Derm Venereol 90: 4–5. , 10 10. Malik M, Gürcan HM & Ahmed AR (2010): Coexistence of mucous membrane pemphigoid and connective-tissue disease.[peertechz.com]
Epistaxis
  • In March 2003 the patient received high doses IVIg but during that treatment, aggravation of the haemorrhagic diathesis with epistaxis, menorrhea and secondary anaemia (Hb 8.2 g/dl) was observed.[springerlink.com]
  • Treatment-related complications included transient hemorrhagic cystitis (1 patient), vaginal bleeding (2 patients), gastrointestinal bleeding (1 patient), epistaxis (1 patient), and antibiotic-responsive febrile neutropenia (all patients).[ncbi.nlm.nih.gov]
  • Severe: – Bleeding episodes—menorrhagia, epistaxis, melena—requiring transfusion or hospitalization - Symptoms interfering seriously with the quality of life 1/25/2015 Idiopathic Thrombocytopenic Purpura Prof.Dr.[slideshare.net]
  • Therefore, our goal is to ameliorate the petechiae and nosebleeds (epistaxis) and not necessarily get one’s hemostatic platelet count up to 100,000.[hopkinsarthritis.org]
  • In the early stages of pregnancy there is generally no need to treat ITP unless the platelet count drops below 10,000-20,000/ul, or there is associated minor bleeding such as epistaxis and bruising.[cancertherapyadvisor.com]

Workup

  • […] future reference, as corticosteroids can change marrow morphology Biopsy is performed before splenectomy to evaluate for possible hypoplasia or fibrosis Unresponsiveness to standard treatment after 6 months is an indication for bone marrow aspiration See Workup[emedicine.medscape.com]
Decreased Platelet Count
  • Additional laboratory evaluation should be performed as clinically indicated, and a trial of discontinuing agents known to decrease platelet counts is recommended.[aafp.org]
  • The resulting shortened life span of platelets in the circulation, together with incomplete compensation by increased platelet production by bone marrow megakaryocytes, results in a decreased platelet count.[emedicine.medscape.com]
HLA-B8
  • The case of a young woman with autoimmune thrombocytopenia, complete IgA deficiency and anti-IgA antibodies is presented and the role of the AH 8-1 supratype (HLA B8, DR3) is discussed.[ncbi.nlm.nih.gov]

Treatment

  • Conservative methods of treatment using steroids, dietotherapy, antibiotics or immunosuppressive agents are not particularly effective in treating fistulas.[ncbi.nlm.nih.gov]

Prognosis

  • Therefore, patients suffering from B-CLL in association with ITP should have a personalized treatment program. 87 Any association of NHL and ITP which changes the prognosis of the patients should be studied carefully.[haematologica.org]
  • Prognosis • Severe bleeding is rare ( 20.[slideshare.net]
  • Immune thrombocytopenia (ITP) in adults: Initial treatment and prognosis. . Accessed March 13, 2019. George JN, et al. Immune thrombocytopenia (ITP) in adults: Second-line and subsequent therapies. . Accessed March 13, 2019. Morrow ES Jr.[mayoclinic.org]
  • Although there is no known cause of ITP and there is no cure, the prognosis for a child with ITP is very good. Usually, the body stops making the antibodies that are attacking the platelets and the disorder resolves on its own.[chop.edu]
  • The fact that somebody has autoimmune thrombocytopenia with a lymphoproliferative disorder is not necessarily a bad sign or prognosis. People with autoimmune thrombocytopenia are often under 40 and female, so a frequent call is to Obstetrics.[hopkinsarthritis.org]

Etiology

  • A greater understanding of the pathogenesis and treatment options can only be definitively clarified by analyses of settings such as the present case, as autoimmune thrombocytopenia is uncommon in patients with malignant lymphoma and the etiology and[ncbi.nlm.nih.gov]
  • No plausible alternative etiology such as drug-mediated thrombocytopenia, marrow failure syndrome or thrombocytopenia related to viral or bacterial infection.[clinicaltrials.gov]
  • Common Etiologies of Thrombocytopenia with Clinical Findings and Suggested Treatment Etiology Severity Clinical presentation Evaluation Treatment Comments Bone marrow suppression by irradiation, chemotherapy, or neoplasia Moderate to severe History of[aafp.org]

Epidemiology

  • Loftus EV, Sandborn WJ (2002) Epidemiology of inflammatory bowel disease. Gastroenterol Clin N Am 31: 1 – 20. ].[peertechz.com]
  • Br J Haematol 128 (5): 698–702 PubMed CrossRef Google Scholar Sloand EM, Klein HG, Banks SM, Vareldzis B, Merritt S, Pierce P (1992) Epidemiology of thrombocytopenia in HIV infection.[link.springer.com]
  • Epidemiology of incident immune thrombocytopenia: a nationwide population-based study in France. Blood. 2014 Nov 20. 124(22):3308-15. [Medline]. Danese MD, Lindquist K, Gleeson M, Deuson R, Mikhael J.[emedicine.medscape.com]
  • "Epidemiology of immune thrombocytopenic purpura in the General Practice Research Database". Br. J. Haematol. 145 (2): 235–44. doi : 10.1111/j.1365-2141.2009.07615.x. PMID 19245432. a b Roback et al. AABB Technical Manual, 16th Ed.[en.wikipedia.org]
Sex distribution
Age distribution

Pathophysiology

  • HCV-associated thrombocytopenia may be considered complex and multifactorial in origin, since different mechanisms have been implicated in its pathophysiology.[ncbi.nlm.nih.gov]
  • ITP (Pathophysiology)(cont.) skperdon.hubpages.com 1/25/2015 Idiopathic Thrombocytopenic Purpura Prof.Dr. Saad S Al Ani Khorfakkan Hospital 7 8.[slideshare.net]
  • Pathophysiology 1. Thrombocytopenia due to a microangiopathic condition requires immediate attention and treatment. Significant advances in understanding the pathophysiology of these processes have been made in the last 10-20 years.[cancertherapyadvisor.com]
  • Idiopathic thrombocytopenic purpura: current concepts in pathophysiology and management. Thromb Haemost. 2008 Jan. 99(1):4-13. [Medline]. [Full Text]. Audia S, Mahévas M, Samson M, Godeau B, Bonnotte B. Pathogenesis of immune thrombocytopenia.[emedicine.medscape.com]

Prevention

  • Bleeding can be prevented if the platelet count can be kept above 10,000 microL.[ncbi.nlm.nih.gov]
  • The National Heart, Lung, and Blood Institute (NHLBI) leads or sponsors many studies aimed at preventing, diagnosing, and treating heart, lung, blood, and sleep disorders.[nhlbi.nih.gov]
  • IVIG may be administered to the mother during the pregnancy or platelet transfusion may be given to the developing fetus to prevent severe bleeding. Cesarean section is recommended for delivery to prevent bleeding. Thanks for your feedback![verywell.com]
  • When treatment is necessary, the two most common forms of treatment are: Steroids, which help prevent bleeding by decreasing the rate of platelet destruction.[chop.edu]

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