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Immune Thrombocytopenic Purpura

ITP

Immune thrombocytopenic purpura (ITP), also called primary immune thrombocytopenia or idiopathic thrombocytopenic purpura, is an autoimmune disease affecting thrombocytes.

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Presentation

The most common presentations observed are bleeding and prolonged bleeding time leading to thrombocytopenia and anemia. Common signs are bleeding spots (purpura) and petechiae mostly on extremities, bleeding gums, epistaxis and menorrhagia. Complications occur due to extremely low blood cell counts (< 5,000 per ul). These include subarachnoid hemorrhage hematomas and lower gastrointestinal bleeding. Care should be taken as ITP patients are at a risk for internal bleeding with slightest trauma. This is unlikely if the platelet count is more than 20,000 per ul. [6].

Easy Bruising
  • An 11-year-old boy presented with epistaxis, petechial hemorrhages, easy bruising, and purpuric rash. He was diagnosed to have immune thrombocytopenic purpura and evidence of concomitant parvovirus B19 and dengue viral infection.[ncbi.nlm.nih.gov]
  • Symptoms can include any of the following: abnormally heavy menstruation, bleeding into the skin, often around the shins, causing a skin rash that looks like pinpoint red spots (petechial rash), easy bruising, nosebleed or bleeding in the mouth.[aarda.org]
  • The result of this is easy bruising or bleeding with no apparent injury. Every year, there is one new case of ITP per 100,000 people. It most often develops in women between the ages of 20 and 40, but it can occur at any age. There is no known cure.[uihc.org]
  • Key points about ITP in children ITP is a blood disorder with decreased blood platelets, which may result in easy bruising, bleeding gums, and internal bleeding.[stlouischildrens.org]
  • A decrease in platelets can result in: Easy bruising Bleeding gums Internal bleeding What Does "Immune Thrombocytopenic Purpura" Mean? ITP is sometimes called by its older name, idiopathic thrombocytopenic purpura.[stanfordhealthcare.org]
Prolonged Bleeding
  • Symptoms Excessive bruising Fatigue Superficial bleeding on the skin Prolonged bleeding Spontaneous bleeding Bleeding Gums Blood in urine or stool Unusually heavy menstrual flow Diagnosis Physical Exam Complete blood count Blood smear Biopsy- Biopsies[raredr.com]
  • The following symptoms require prompt attention: Bleeding symptoms easy bruising bleeding for no apparent reason unusually heavy or prolonged bleeding petechiae , or tiny areas of pinpoint bleeding on the skin of the arms or legs blood in the urine or[uihc.org]
  • bleeding from cuts profuse bleeding during surgery Some people with ITP have no symptoms.[healthline.com]
  • Prolonged bleeding from the gums. Blood in the poo (stools) or urine. A heavy blow to the head, especially if the child is unwell afterwards in any way. Persistent or severe headache. Sickness (vomiting). Unexplained drowsiness.[patient.info]
Splenomegaly
  • Clinical examination revealed mucosal hemorrhages and splenomegaly. The initial diagnosis was immune thrombocytopenic purpura (ITP) and he received intravenous gamma globulins and steroids with good hematologic and clinical response.[ncbi.nlm.nih.gov]
  • There is no evidence of splenomegaly on abdominal exam. A complete blood count is significant for a platelet count of 24,000/μL and peripheral blood smear demonstrates enlarged platelets. He is started on corticosteroids.[medbullets.com]
  • Splenomegaly is also a typical finding in ALPS and leukemia. The combination of thrombocytopenia and splenomegaly would also suggest hypersplenism from liver disease and/or portal hypertension. Atypical Laboratory Findings: See below.[clinicaladvisor.com]
  • Hepatomegaly, splenomegaly and lympadenopathy were absent and ITP diagnose was verified.[benthamopen.com]
  • In an adult, hepatosplenomegaly is also atypical for ITP and may indicate chronic liver and other diseases; in fact, splenomegaly excludes the diagnosis of ITP.[emedicine.medscape.com]
Anemia
  • Mild cases of ADAMTS13 deficiency may be initially confused with immune thrombocytopenic purpura if hemolytic anemia is not severe and renal or neurological symptoms are not present.[ncbi.nlm.nih.gov]
  • Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia[ncbi.nlm.nih.gov]
  • Hematogones (HGs) are benign immature B cells in bone marrow with a variety of benign and malignant conditions, including idiopathic thrombocytopenic purpura, leukemia, lymphoma, red blood cell aplasia, iron deficiency anemia, amegakaryocytosis, regenerative[ncbi.nlm.nih.gov]
  • […] reported a rare case with late-onset ITP after withdrawal of PEG-IFN treatment.A 53-year-old male with hepatitis C developed massive gum bleeding and a severe, reversible, immune thrombocytopenia 2 weeks after cessation of PEG-IFN treatment for HCV due to anemia[ncbi.nlm.nih.gov]
  • A complete blood count shows thrombocytopenia and low hemoglobin suggests anemia due to blood loss. Blood smear is mandatory to evaluate and rule out other causes of thrombocytopenia.[symptoma.com]
Fever
  • Here, we describe a previously healthy adolescent who presented with fever, influenza-like symptoms and acute onset of generalized petechiae and active oral mucosa bleeding on the third day of his illness.[ncbi.nlm.nih.gov]
  • Most costly AEs were urinary tract infection, aseptic meningitis, and fever ( 5000 /case); headache, nasal congestion, and hemolytic event were 4,000-5,000/case. Cost per AE did not differ by treatment.[ncbi.nlm.nih.gov]
  • Common side effects may include fever, chills, headache, light-headedness, flushing, itching, and a change in blood pressure. Anti-D IgG or WinRho, given intravenously.[cincinnatichildrens.org]
  • Symptoms to ask about include: gingival bleeding gastrointestinal bleeding menorrhagia neurological symptoms (intracerebral bleeding) a relapsing-remitting course mild fevers left upper quadrant discomfort due to splenic enlargement bleeding following[sharinginhealth.ca]
  • Possible side effects include nausea, vomiting, headaches, or fever. In rare cases, aseptic meningitis or kidney failure has occurred. Anti D immune globulin (WinRho).[childrensmn.org]
Constitutional Symptom
  • Constitutional symptoms and reticuloendothelial manifestations (p   .05), as well as pericarditis (25% vs. 10%, p   .029) and central nervous system (CNS) involvement (30% vs. 14%, p   .029) were more common in cSLE.[ncbi.nlm.nih.gov]
Bleeding Gums
  • Key points about ITP in children ITP is a blood disorder with decreased blood platelets, which may result in easy bruising, bleeding gums, and internal bleeding.[stlouischildrens.org]
  • A decrease in platelets can result in: Easy bruising Bleeding gums Internal bleeding What Does "Immune Thrombocytopenic Purpura" Mean? ITP is sometimes called by its older name, idiopathic thrombocytopenic purpura.[stanfordhealthcare.org]
  • 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]
  • Common signs are bleeding spots (purpura) and petechiae mostly on extremities, bleeding gums, epistaxis and menorrhagia. Complications occur due to extremely low blood cell counts ( 5,000 per ul).[symptoma.com]
  • Symptoms Excessive bruising Fatigue Superficial bleeding on the skin Prolonged bleeding Spontaneous bleeding Bleeding Gums Blood in urine or stool Unusually heavy menstrual flow Diagnosis Physical Exam Complete blood count Blood smear Biopsy- Biopsies[raredr.com]
Oral Bleeding
  • In some instances there is oral bleeding, epistaxis, rectal bleeding or haematuria. Morbidity in ITP is usually minimal and parents need to be reassured of this.[rch.org.au]
  • Oral bleeding ("wet purpura") has historically been considered to be a risk factor for more serious bleeding such as intracranial hemorrhage, although there are insufficient data to prove this.[clinicaladvisor.com]
Purpura
  • There were 5472 subjects aged 1-84 years with a new diagnosis of immune thrombocytopenic purpura as the purpura group since 1998-2010 and 21,887 sex-matched and age-matched, randomly selected subjects without immune thrombocytopenic purpura as the non-purpura[ncbi.nlm.nih.gov]
  • Immune thrombocytopenic purpura may be associated with meningococcal vaccination in adulthood. Responses to treatment in immune thrombocytopenic purpura vary among patients.[ncbi.nlm.nih.gov]
  • For immune thrombocytopenic purpura, the patient was started on the thrombopoietin receptor agonist romiplostim nine months ago.[ncbi.nlm.nih.gov]
  • Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis. J Pediatr Hematol Oncol . 2003 Aug. 25(8):660-4. [Medline] . Fogarty PF, Segal JB. The epidemiology of immune thrombocytopenic purpura.[emedicine.medscape.com]
  • Practice Essentials Immune thrombocytopenic purpura (ITP) is a clinical syndrome in which a decreased number of circulating platelets (thrombocytopenia) (see the image below) manifests as a bleeding tendency, easy bruising (purpura), or extravasation[emedicine.medscape.com]
Petechiae
  • In this retrospective cohort study conducted in 63 children with idiopathic thrombocytopenic purpura (ITP) in China; petechiae, bruises and bleeding were the major presentations. Most cases required therapy with one/more treatment options.[ncbi.nlm.nih.gov]
  • Here, we describe a previously healthy adolescent who presented with fever, influenza-like symptoms and acute onset of generalized petechiae and active oral mucosa bleeding on the third day of his illness.[ncbi.nlm.nih.gov]
  • Clinical manifestations may range from spontaneous formation of purpura and petechiae, especially on the extremities, to epistaxis, bleeding at the gums or menorrhagia, any of which occur usually if the platelet count is below 20,000 per μl.[ncbi.nlm.nih.gov]
  • Petechiae, purpura, vesicles, bullae, target lesions, desquamation). ITP is one of those entities that we often consider when we are evaluating a rash.[pedemmorsels.com]
  • Bleeding into the skin takes the form of purpura or petechiae. Purpura is a purplish or reddish-brown rash or discoloration of the skin; petechiae are small round pinpoint hemorrhages.[medical-dictionary.thefreedictionary.com]
Epistaxis
  • An 11-year-old boy presented with epistaxis, petechial hemorrhages, easy bruising, and purpuric rash. He was diagnosed to have immune thrombocytopenic purpura and evidence of concomitant parvovirus B19 and dengue viral infection.[ncbi.nlm.nih.gov]
  • Clinical manifestations may range from spontaneous formation of purpura and petechiae, especially on the extremities, to epistaxis, bleeding at the gums or menorrhagia, any of which occur usually if the platelet count is below 20,000 per μl.[ncbi.nlm.nih.gov]
  • A 32-year-old Persian White man visited his private hematologist complaining of recent episodes of epistaxis and appearance of petechial lesions 24 hours after receiving a meningococcal vaccine.[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]
  • Common signs are bleeding spots (purpura) and petechiae mostly on extremities, bleeding gums, epistaxis and menorrhagia. Complications occur due to extremely low blood cell counts ( 5,000 per ul).[symptoma.com]
Guillain-Barré Syndrome
  • Guillain-Barré syndrome (GBS) is a potentially life-threatening autoimmune disease causing demyelination of peripheral nerves. Multiple variants of GBS exist, with acute motor and sensory axonal neuropathy (AMSAN) being the most severe.[ncbi.nlm.nih.gov]

Workup

Diagnosis is partly made through a careful history and nature of symptoms. A complete blood count shows thrombocytopenia and low hemoglobin suggests anemia due to blood loss. Blood smear is mandatory to evaluate and rule out other causes of thrombocytopenia.

Congenital thrombocytopenia shows abnormal morphology in leucocytes and erythrocytes as well, suggesting an underlying bone marrow disease. A blood smear shows large number of schistocytes suggesting thrombocytopenia due to thrombotic purpura or hemolytic uremic syndrome.

A bone marrow examination is done in cases who do not respond to the primary treatment. The bone marrow biopsy may show reduced counts of megakaryocytes that help in diagnosis of ITP. Also, leukemia is ruled out. In cases where splenectomy is done, bone marrow examination is done to evaluate for fibrosis.

Decreased Platelet Count
  • platelet count and the patient is bleeding, purpura. 05:54 Or let's say that your patient is deficient of IIb/IIIa. 05:59 If you're deficient of IIb/IIIa, this brings us to a condition called Glanzmann's thrombasthenia. 06:05 What are the tests that[lecturio.com]
  • 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]

Treatment

Treatment aims at increasing the platelet level to a safe value of more than 30x 109/L and maintaining the count thereafter till remission. Corticosteroids comprise the first line of treatment that includes prednisone, dexamethasone and methylprednisolone. In case of bleeding tendencies, along with corticosteroids anti-RhD injections are also given to stimulate the platelet count. Studies show that the platelet value increases by > 50 × 109/L in a few days to several weeks after giving prednisolone. Patients requiring anti–RhD treatment are first screened direct antiglobulin test and the Rh type is determined before initiating the treatment. The standard dose is 50 nanogms/kg. The response is seen after 48- 72 hrs.

Splenectomy is the most commonly done as a part of treatment especially in chronic ITPs. The procedure has been curative to majority of patient, as it lowers the rate of relapse [7].

Prognosis

Prognosis of acute ITP in children is good. Studies showed that about 80% of children recovered completely. There might be a relapse after 6 weeks and only about a small percentage of children develop a chronic disease. Chronic disease is usually seen in adults. Patients gain a normal platelet count with prednisone treatment. If the prednisone is reduced chances of platelet reducing further increases.

Etiology

The exact cause of the immune mediated antibodies that cause platelet destruction is unknown, however it is postulated that a reduction in suppressor T regulatory cells and increase in B cell activating factor may be responsible for the generation of these autoantibodies. Certain theories relate activation of self-reactive platelet antibodies after infections such as HIV, H pylori and hepatitis C. Here the antibodies cross reacts with the platelets leading to immune thrombocytopenic purpura [2].

Epidemiology

The incidence of ITP is estimated to be 50-100 cases per million per year [3]. It is accounted that most of the adult age group is affected with this disease, children being concerned 50% off the adult ratio. The mortality rate is higher in adults. It was concluded that over 90% deaths occurred in the age group of individuals over 45 years [4].

Sex distribution
Age distribution

Pathophysiology

Dr. William Harrington had first experimented on himself, along with his other colleagues by injecting with the blood of patients with ITP. The experiment resulted into severe thrombocytopenia in their blood sample lasting for several days. Immune thrombocytopenic purpura involves both cellular and humoral immunity. There are certain autoantibodies found which are directed against the platelet surface glycoprotein (GP) complexes gp IIb-IIIa [5] [2].

The autoantibodies are mostly of IgG subtype and they attach to the targeted platelets on their surfaces. It was found that in ITP patients who were injected with in-labeled autologous platelets, the uptake is primarily from the spleen and liver. After platelets are attached to the antibodies’ surface, there is degradation and macrophages secrete cytokines that stimulate CD4 T cell clones. These CD4 T cells identifies distinct epitopes on gp IIb-IIIa, leading to a general autoimmune response and massive platelet destruction. The trigger for such a response is not known. Studies show that platelet associated auto antibodies are found in about 50-70% affected individuals.

B cells in ITP produce antibodies directed against the normal platelet antigens thereby initiating response from the CD4 T cells. A potent immunosuppressive cytokine called beta 1, is found to be elevated in patients with ITP in remission as compared to those with active ITP. The autoreactive T cells are found in healthy individuals, but the active form is only found in patients with autoimmune diseases. Also, along with destruction of platelets there is suppression of production of megakaryocytes leading to thrombocytopenia.

Prevention

Since the exact cause is not known, there is no specific way to prevent this disorder. However, the complications can be prevented and special precautions are to be observed to prevent bleeding injuries.

Summary

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder also referred to as primary immune thrombocytopenia or idiopathic thrombocytopenic purpura, characterized by destruction of platelets and impairment of platelet production (thrombopoeisis) leading to a tendency to prolonged bleeding [1].

Patient Information

Immune thrombocytopenic purpura (ITP), also called primary immune thrombocytopenia or idiopathic thrombocytopenic purpura, is an autoimmune mediated disorder wherein antibodies are directed to destroy or kill normal platelets leading to bleeding disorders. The cause is not known. Certain drugs and infections such as HIV and Helicobacter pylori infections are thought to be some of the causes for this disease. Signs and symptoms include bleeding from gums, nostrils, bruises on extremities, easy bleeding to slightest injury and there is a increased risk of intracranial hemorrhage which may prove fatal. Treatment is aimed in restoring the platelet values to normal levels by treating with corticosteroids. Chronic long standing patients are advised spleen removal (splenectomy) that has shown considerable results.

References

Article

  1. Arnold DM, Patriquin C, Toltl LJ, et al. Diseases of platelet number: immune thrombocytopenia, neonatal all immune thrombocytopenia, and post transfusion purpura. In: Hoffman R, Benz EJ Jr, Silberstein LE, et al., eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2012: chap 133.
  2. Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood.2010 Jan 14;115(2):168–186.
  3. Watts RG. Idiopathic thrombocytopenic purpura: a 10-year natural history study at the children's hospital of Alabama. Clinical pediatrics 2004 43(8): 691–702.
  4. Schoonen WM, Kucera G, Coalson J, et al. Epidemiology of immune thrombocytopenic purpura in the General Practice Research Database. Br. J. Haematol. 2009 145(2): 235–44.
  5. Cines DB, Bussel JB, Liebman HA, Luning Prak ET. The Immune Thrombocytopenic Purpura syndrome: pathogenic and clinical diversity. Blood. 2009 Jun 25; 113(26):6511–6521.
  6. Bennett CM, Tarantino M. Chronic thrombocytopenia in children: epidemiology and clinical presentation. Hematol Oncol Clin North Am 2009 23(6):1223–1238
  7. Cines DB, McMillan R. Management of adult idiopathic thrombocytopenic purpura. Annu Rev Med. 2005;56:425-42.

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Last updated: 2018-06-21 18:47