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Encyclopedia > Thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Classification & external resources
ICD-10 M31.1
ICD-9 446.6
DiseasesDB 13052
MedlinePlus 000552
eMedicine emerg/579  neuro/499 med/2265
MeSH D011697

Thrombotic thrombocytopenic purpura (TTP or Moschcowitz disease) is a rare disorder of the blood coagulation system. Most cases of TTP arise from deficiency or inhibition of the enzyme ADAMTS13, which is responsible for cleaving large multimers of von Willebrand factor.[1] This leads to hemolysis and end-organ damage, and may require plasmapheresis therapy. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // M00-M99 - Diseases of the musculoskeletal system and connective tissue (M00-M25) Arthropathies (M00-M03) Infectious arthropathies (M00) Pyogenic arthritis (M01) Direct infections of joint in infectious and parasitic diseases classified elsewhere (M02) Reactive arthropathies (M023) Reiters disease (M03) Postinfective and reactive arthropathies in diseases classified elsewhere (M05-M14... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... A rare disease (sometimes known as an orphan disease) has such a low prevalence in a population that a doctor in a busy general practice would not expect to see more than one case a year. ... Coagulation is a complex process by which blood forms solid clots. ... Ribbon diagram of the enzyme TIM, surrounded by the space-filling model of the protein. ... ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13) is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting. ... Von Willebrand factor is a blood glycoprotein of the coagulation system. ... {{otheruses4|1=medical hemoglobin]] into the surrounding fluid (plasma, in vivo). ... Plasmapheresis (from the Greek plasma, something molded, and apheresis, taking away) is the removal, treatment, and return of (components of) blood plasma from blood circulation. ...

Contents

Signs and symptoms

Classically, the following five symptoms are indicative of this elusive disease:

A patient may notice dark urine from the hemolytic anemia. Because of the many small areas of ischemia produced by clots in the microvasculature, symptoms may be diffuse and fluctuating, including the classical bruising, confusion, or headache, but also nausea and vomiting (from ischemia in the GI tract or from central nervous system involvement), chest pain from cardiac ischemia, seizures, muscle and joint pain, etc.[citation needed] Neurology is a branch of medicine dealing with disorders of the nervous system. ... For other uses, see Stroke (disambiguation). ... A headache (cephalalgia in medical terminology) is a condition of pain in the head; sometimes neck or upper back pain may also be interpreted as a headache. ... Acute renal failure (ARF) is a rapid loss of renal function due to damage to the kidneys, resulting in retention of nitrogenous (urea and creatinine) and non-nitrogenous waste products that are normally excreted by the kidney. ... This article needs additional references or sources for verification. ... Thrombocytopenia (or -paenia, or thrombopenia in short) is the presence of relatively few platelets in blood. ... A 250 ml bag of newly collected platelets. ... A bruise or contusion or ecchymoses is a kind of injury, usually caused by blunt impact, in which the capillaries are damaged, allowing blood to seep into the surrounding tissue. ... Purple discolorations on the skin caused by bleeding underneath the skin. ... In medicine (hematology) microangiopathic hemolytic anemia (MAHA) is a subgroup of hemolytic anemia (anemia, loss of red blood cells through destruction) caused by factors in the small blood vessels. ... This article discusses the medical condition. ... Blood films, Giemsa stained A blood film or peripheral blood smear is a slide made from a drop of blood, that allows the cells to be examined. ...


Causes

TTP, as with other microangiopathic hemolytic anemias (MAHAs), is caused by spontaneous aggregation of platelets and activation of coagulation in the small blood vessels. Platelets are consumed in the coagulation process, and the fibrin mesh quite literally "tears apart" red blood cells, leading to hemolysis. In medicine (hematology) microangiopathic hemolytic anemia (MAHA) is a subgroup of hemolytic anemia (anemia, loss of red blood cells through destruction) caused by factors in the small blood vessels. ... Look up Aggregation in Wiktionary, the free dictionary The term aggregation may refer to— in economics, combining entities into a single entity which represent them, like aggregation of individual demand to total, or market, demand. ... A 250 ml bag of newly collected platelets. ... Coagulation is a complex process by which blood forms solid clots. ... f you all The blood vessels are part of the circulatory system and function to transport blood throughout the body. ... Fibrin is a protein involved in the clotting of blood. ... “Red cell” redirects here. ... {{otheruses4|1=medical hemoglobin]] into the surrounding fluid (plasma, in vivo). ...


Roughly, there are two forms of TTP: idiopathic and secondary TTP. A special case is the inherited deficiency of ADAMTS13, known as the Upshaw-Schulman syndrome.


Idiopathic TTP

The idiopathic form of TTP was recently linked to the inhibition of the enzyme ADAMTS13 by antibodies, rendering TTP an autoimmune disease. ADAMTS13 is a metalloproteinase responsible for the breakdown of the so-called von Willebrand factor (vWF), a protein that links platelets, blood clots, and the blood vessel wall in the process of blood coagulation. Very large vWF molecules are more prone to lead to coagulation. Hence, without proper cleavage of vWF by ADAMTS13, coagulation occurs at a higher rate, especially in the part of the blood vessel system where vWF is most active due to high shear stress: in the microvasculature. Idiopathic means arising spontaneously or from an obscure or unknown cause. ... Ribbon diagram of the enzyme TIM, surrounded by the space-filling model of the protein. ... Each antibody binds to a specific antigen; an interaction similar to a lock and key. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... The metalloendopeptidases (also called metalloproteinases or metalloproteases) are a class of enzymes from the group of endopeptidases. ... Von Willebrand factor is a blood glycoprotein of the coagulation system. ... A thrombus is the final product of blood coagulation, through the aggregation of platelets and the activation of the humoral coagulation system. ... Coagulation is a complex process by which blood forms solid clots. ... Shear stress is a stress state where the stress is parallel or tangential to a face of the material, as opposed to normal stress when the stress is perpendicular to the face. ...


In idiopathic TTP, severely decreased (<5% of normal) ADAMTS13 activity can be detected in most (80%) patients, and inhibitors are often found in this subgroup (44-56%).


Secondary TTP

Secondary TTP is diagnosed when the patient's history mentions one of the known features associated with TTP. It comprises about 40% of all cases of TTP. Predisposing factors are:

The mechanism of secondary TTP is poorly understood, as ADAMTS13 activity is generally not as depressed as in idiopathic TTP, and inihibitors cannot be detected. Cancer is a class of diseases or disorders characterized by uncontrolled division of cells and the ability of these to spread, either by direct growth into adjacent tissue through invasion, or by implantation into distant sites by metastasis (where cancer cells are transported through the bloodstream or lymphatic system). ... Bone marrow transplantation is a medical procedure that involves stem cell transplantation. ... This article is about human pregnancy in biological females. ... This article does not cite any references or sources. ... Ticlopidine is a drug used for its antiplatelet action. ... A box of Plavix Clopidogrel is a potent oral antiplatelet agent often used in the treatment of coronary artery disease, peripheral vascular disease, and cerebrovascular disease. ... Cyclosporine (IPA: ), ciclosporin (INN), or cyclosporin (former BAN), is an immunosuppressant drug. ... Mitomycin or mitomycin C is a form of chemotherapy given intravenously to treat upper gastro-intestinal (e. ... Tacrolimus (also FK-506 or Fujimycin) is a 23-membered macrolide lactone discovered in 1984 from the fermentation broth of a Japanese soil sample that contained the bacteria Streptomyces tsukubaensis. ... Interferons (IFNs) are natural proteins produced by the cells of the immune system of most vertebrates in response to challenges by foreign agents such as viruses, bacteria, parasites and tumor cells. ... The human immunodeficiency virus (HIV) is a frequently mutating retrovirus that attacks the human immune system and which has been shown to cause acquired immune deficiency syndrome (AIDS). ...


Upshaw-Schulman syndrome

A hereditary form of TTP is called the Upshaw-Schulman syndrome; this is generally due to inherited deficiency of ADAMTS13 (frameshift and point mutations). Patients with this inherited ADAMTS13 deficiency have a surprisingly mild phenotype, but develop TTP in clinical situations with increased von Willebrand factor levels, e.g. infection. Reportedly, 5-10% of all TTP cases are due to Upshaw-Schulman syndrome. Von Willebrand factor is a blood glycoprotein of the coagulation system. ...


Treatment

Since the early 1990s, plasmapheresis has become the treatment of choice for TTP.[2] This is an exchange transfusion involving removal of the patient's blood plasma through apheresis and replacement with donor plasma (fresh frozen plasma or cryosupernatant); the procedure has to be repeated daily to eliminate the inhibitor and ablate the symptoms. Lactate dehydrogenase levels are generally used to monitor disease activity. Plasmapheresis may need to be continued for 1-8 weeks before patients with idiopathic TTP cease to consume platelets and begin to normalize their hemoglobin. No single laboratory test (platelet count, LDH, ADAMTS13 level, or inhibitory factor) is indicative of recovery; research protocols have used improvement or normalization of LDH as a measure for ending plasmapheresis. Although patients may be critically ill with failure of multiple organ systems during the acute illness, including renal failure, myocardial ischemia, and neurologic symptoms, recovery over several months may be complete in the absence of a frank myocardial infarct, stroke, or CNS hemorrhage.[citation needed] For the band, see 1990s (band). ... Plasmapheresis (from the Greek plasma, something molded, and apheresis, taking away) is the removal, treatment, and return of (components of) blood plasma from blood circulation. ... An exchange transfusion is a medical treatment in which apheresis is used to remove one persons red blood cells or platelets and replace them with transfused blood products. ... Blood plasma is the liquid component of blood, in which the blood cells are suspended. ... Whole blood enters the centrifuge on the left and separates into layers so that selected components can be drawn off on the right. ... Categories: Possible copyright violations ... Lactate dehydrogenase (LDH) is an enzyme (EC 1. ...


Many TTP patients need additional immunosuppressive therapy, with glucocorticoid steroids (e.g. prednisolone or prednisone), vincristine, cyclophosphamide, splenectomy or a combination of the above. Rituximab, a monoclonal antibody targeting B cells, has been successfully used to treat patients with refractory disease. Immunosuppression is the medical suppression of the immune system. ... Glucocorticoids are a class of steroid hormones characterised by an ability to bind with the cortisol receptor and trigger similar effects. ... This article is about the chemical family of steroids. ... Prednisolone is the active metabolite of prednisone. ... Prednisone is a synthetic corticosteroid drug which is usually taken orally but can be delivered by intramuscular injection and can be used for a great number of different conditions. ... Vincristine (Oncovin®) is a vinca alkaloid from the Madagascar periwinkle (Catharanthus roseus, formerly Vinca rosea and hence its name). ... Cyclophosphamide (the generic name for Cytoxan, Neosar) is a nitrogen mustard alkylating agent, used to treat various types of cancer and some autoimmune disorders. ... A splenectomy is a procedure that involves the removal of the spleen by operative means. ... Rituximab, sold under the trade names Rituxan® and MabThera®, is a monoclonal antibody used in the treatment of B cell non-Hodgkins lymphoma, B cell leukemia, and some autoimmune disorders. ... Monoclonal antibodies (mAb) are antibodies that are identical because they were produced by one type of immune cell, all clones of a single parent cell. ... B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ...


Children with Upshaw-Schulman syndrome receive plasma every three weeks prophylactically; this maintains adequate levels of functioning ADAMTS13.


Epidemiology

The incidence of TTP is about 4-6 per million people per year. As with most other autoimmune disorders, idiopathic TTP occurs more often in women and blacks, while the secondary forms do not show this distribution. Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ...


Prognosis

The mortality rate is approximately 95% for untreated cases, but the prognosis is reasonably favorable (80-90%) for patients with idiopathic TTP diagnosed and treated early with plasmapheresis.


Approximately one-third of patients experiencing a TTP episode have a relapse within 10 years following their first attack.


Secondary TTP still has a dismal prognosis, with mortality rates despite treatment being reported as 59% to 100%.


History

TTP was initially described in a 16-year old girl by Dr Eli Moschcowitz of New York City in 1924.[3] Moschcowitz ascribed the disease (incorrectly) to a toxic cause. Moschcowitz noted that his 16 year-old patient had anemia; petechiae; microscopic hematuria; and at autopsy, disseminated microvascular thrombi. Since that time, the pathophysiology, etiology, and medical management of TTP has expanded. New York, New York and NYC redirect here. ...


See also

Plasmapheresis (from the Greek plasma, something molded, and apheresis, taking away) is the removal, treatment, and return of (components of) blood plasma from blood circulation. ... An exchange transfusion is a medical treatment in which apheresis is used to remove one persons red blood cells or platelets and replace them with transfused blood products. ... ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13) is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting. ...

References

  1. ^ Moake JL. Von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. Semin Hematol 2004;41:4-14. PMID 14727254.
  2. ^ Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood 2004;103:4043-9. PMID 14982878.
  3. ^ Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc NY Pathol Soc 1924;24:21-4. Reprinted in: Mt Sinai J Med 2003;70:352-5. PMID 14631522.
  • Moake JL. Thrombotic microangiopathies. N Engl J Med 2003;347:589-600. PMID 12192020.

The New England Journal of Medicine (NEJM) is a peer-reviewed medical journal published by the Massachusetts Medical Society. ...

External links

  • Thrombotic thrombocytopenic purpura
  • TTPNetwork Patient Support Group

  Results from FactBites:
 
Thrombotic Thromboctyopenic Purpura (823 words)
Thrombotic thrombocytopenic purpura (TTP) is a rare blood condition characterized by the formation of small clots (thrombi) within the circulation, which results in the consumption of platelets and thus a low platelet count (thrombocytopenia).
The first case of TTP was described by Moschcowitz, in 1925, in which a 16 year old female was admitted to the hospital with an acute and fatal illness that included stroke and heart failure.
Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome were once thought to represent opposite ends of the spectrum of the same disease process and are classified by some as thrombotic microangiopathy (TMA).
  More results at FactBites »

 
 

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