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].

• Splenomegaly

  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]

  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]

  […] the platelet membrane, not the bone marrow. 03:55 Treatment maybe IVIg and if counts are less than 50,000. 04:01 Remember, normal, lower limit, is at least 150,000, so that's pretty significant drop. 04:06 And you may have the spleen which may undergo splenomegaly [lecturio.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]

  Keywords polycythemia vera, immune thrombocytopenic purpura, treatment method Polycythemia Vera (PV) is a chronic, clonal and progressive myeloproliferative disease generally characterized by leukocytosis, thrombocytosis, splenomegaly accompanied by an [oatext.com]

• 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]

  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. Also called idiopathic thrombocytopenic purpura, immune thrombocytopenia, and ITP. [cancer.gov]

  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]

  Snapshot A 42-year-old man presents to his primary care physician's office for easy bruising and gingival bleeding. He reports his symptoms began one day prior to presentation and has never occurred before. [medbullets.com]

• Prolonged Bleeding

  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. [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]

  bleeding from the cuts, we talk about increase in bleeding time, spontaneous bleeding from the gums, bleeding in to the urine or stool, and if it's a lady, when she's then having her menses, she may have increase menstrual flow. 02:38 So now you should [lecturio.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]

• Anemia

  If there is anemia out of proportion to the amount of bleeding, a reticulocyte count should be performed. A low reticulocyte count would suggest lack of production as seen in leukemia and aplastic anemia. [clinicaladvisor.com]

  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]

  All 10 patients received rituximab 2-18 days after diagnosis, and corticosteroids combined with rituximab within MeSH terms, Substances, Grant support MeSH terms Adolescent Adrenal Cortex Hormones/therapeutic use Adult Aged Anemia, Hemolytic, Autoimmune [ncbi.nlm.nih.gov]

  Orofacial manifestations of hematological disorders anemia and hemostatic disorders. Indian J Dent Res 2011; 22: 454-61. ]. [benthamopen.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]

  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]

  Fever and infection are infrequently presenting features of ITP as addressed above. [clinicaladvisor.com]

• Goiter

  A 9-year-old girl presented with ITP and features of hypothyroidism in the form of goiter and growth retardation. She was subsequently found to have RTH. [ncbi.nlm.nih.gov]

• Bleeding Gums

  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]

  Platelets help stop bleeding. So, a decrease in platelets can result in easy bruising, bleeding gums, and bleeding inside the body. The lower the platelet count, the greater the risk of bleeding. [stanfordchildrens.org]

  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. [symptoma.com]

  Tiny red dots under the skin that are due to very small bleeds. Nosebleeds Bleeding in your mouth, or in and around your gums Heavy menstrual periods Blood in your vomit, urine, or stool Bleeding in your head. [urmc.rochester.edu]

• 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]

  All patients with oral mucosal blistering had a history of oral bleeding and had bleeding at the time of enrollment. Most bleeding events occurred during the post-treatment period or in patients who did not have a response to the study drug. [doi.org]

• Arthritis

  Thyroid diseases, type 1 diabetes, rheumatoid arthritis, psoriasis and inflammatory bowel disease are some examples. [news-medical.net]

  The risk appears to be higher in people who also have diseases such as rheumatoid arthritis, lupus and antiphospholipid syndrome. Complications A rare complication of immune thrombocytopenia is bleeding into the brain, which can be fatal. [mayoclinic.org]

  Pregnancy Immune disorders, such as rheumatoid arthritis and lupus Low-grade lymphomas and leukemias may make abnormal antibodies that attack platelets. Sometimes the cause of ITP is not known. [urmc.rochester.edu]

  […] immunomodulation in adults patients with immune thrombocytopenic purpura sk PNR obnovený, ale rezervy en Idiopathic thrombocytopenic purpura sk Tieňovaná časť sa nazýva Skúšobná plocha en ra including thyroid disorders, systemic lupus erythematosus, rheumatoid arthritis [sk.glosbe.com]

• Contusion

  Phase 2 The most frequently reported adverse events in phase 2 were contusions, ecchymosis, or both (occurring in 59% of the patients in the AMG 531 groups and 75% of those in the placebo group); epistaxis (41% and 50%, respectively); mild-to-moderate [doi.org]

• Purpura

  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]

  Immune thrombocytopenic purpura Other names Idiopathic thrombocytopenic purpura, idiopathic immune thrombocytopenia, primary immune thrombocytopenia, idiopathic thrombocytopenic purpura, primary immune thrombocytopenic purpura, autoimmune thrombocytopenic [en.wikipedia.org]

  ITP 1/25/2015 Idiopathic Thrombocytopenic Purpura Prof.Dr. [slideshare.net]

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

• Petechiae

  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]

  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]

  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]

  Petechiae on the lower extremities Oral petechiae/purpura - lower lip Petechia on the tongue in a person with platelets of 3 due to ITP Petechia of the lower leg in a person with platelets of 3 due to ITP Pathogenesis[edit] In approximately 60 percent [en.wikipedia.org]

• Epistaxis

  BACKGROUND: Idiopathic thrombocytopenic purpura is characterized by extravasation of blood into the subcutaneous tissue, mucous membranes or skin; it generates bleeding manifestations as ecchymotic lesions, petechiae of sudden onset, epistaxis, bleeding [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]

• Hematuria

  Case Presentation We report a case of a 29 year-old male who was presented with immune thrombocytopenia-induced hemoptysis, macroscopic hematuria and generalized petechiae. [ann-clinmicrob.biomedcentral.com]

  Gastrointestinal bleeding, hematuria, and intracranial hemorrhage are rare and are typically seen in older patients with severe ( Patients will have a normal CBC except for thrombocytopenia, a normal peripheral smear, and PT/PTT. [journals.lww.com]

  Severe visceral bleeding (hematuria, gastrointestinal hemorrhage or cerebromeningeal bleeding) is mainly observed when thrombocytopenia is below 10,000/microL. [orpha.net]

• Uremia

  Additional factors (e.g., age, lifestyle, uremia) affect the risk and should be evaluated before the appropriate management is determined ( 6, 7 ). [nature.com]

  The severity of thrombocytopenia correlates to some extent but not completely with the bleeding risk. 7, 11 Additional factors (eg, age, lifestyle factors, uremia) affect the risk and should be evaluated before the appropriate management is determined [doi.org]

• Intracranial Hemorrhage

  Life-threatening hemorrhage (intracranial hemorrhage) complicates acute ITP 4. [slideshare.net]

  This includes, but is not limited to, life threatening gastrointestinal bleeding and intracranial hemorrhage. [emdocs.net]

  […] trauma–induced intracranial hemorrhage is the most frequent cause of death in association with ITP. [emedicine.medscape.com]

  Severe headache occurring while the patient is still thrombocytopenic often leads to head CT scan to rule out intracranial hemorrhage. [clinicaladvisor.com]

  Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis. J Pediatr Hematol Oncol. 2003 ;25: 660 - 664. Google Scholar | Crossref | Medline | ISI Medeiros D, Buchanan GR. [doi.org]

• 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]

  […] rozpočtových prostriedkoch en They include patients with idiopathic thrombocytopenic purpura (ITP) who do not have enough platelets (components in the blood that help it to clot), and who are at high risk of bleeding, and patients with certain diseases (Guillain-Barré [sk.glosbe.com]

  […] cells called neutrophils en They include patients with idiopathic thrombocytopenic purpura (ITP) who do not have enough platelets (components in the blood that help it to clot), and who are at high risk of bleeding, and patients with certain diseases (Guillain-Barré [sl.glosbe.com]

  […] and 23 months after rituximab infusions, respectively; colon cancer was diagnosed in a 63-year-old man 3 months after rituximab infusions; a 75-year-old woman developed pancreatic cancer 6 months after rituximab infusions; another patient developed Guillain-Barré [doi.org]

• Paresis

  • Pentad of Findings in Thrombotic Thrombocytopenic Purpura (TTP) • 1- Microangiopathic hemolytic anemia • 2 -Thrombocytopenia • 3 -Neurologic abnormalitiesconfusion, headache, paresis, visual hallucinations, seizures • 4 -Fever • 5 -Renal dysfunction [slideshare.net]

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

  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]

  Results The literature synthesis and themes from the focus group data suggest that decreased platelet counts, disease symptoms, and treatment side effects influence multiple domains of HRQoL for ITP patients. [hqlo.biomedcentral.com]

• Hyperglobulinemia

  It should be noted that many hematological diseases have oral manifestations like ITP: aplastic anemia, Glanzman thrombasthenia, Hemophilias, Ehlers Danlos Syndrome, Hyperglobulinemia and von Willebrand disease [ 19 Nakhoul IN, Kozuch P, Varma M. [benthamopen.com]

• Hemoglobin Decreased

  Response to Initial Anti-D Treatment: Platelet Increase and Hemoglobin Decrease Table 2. [bloodjournal.hematologylibrary.org]

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 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.

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].

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].

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.

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.

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].

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.

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.