Disorders Of Primary Hemostasis (Quantitative Platelet Disorder)

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A relatively uncommon phenomenon caused by ex vivo agglutination of platelets.

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Pseudothrombocytopenia

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Pseudothrombocytopenia is also known as?

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Spurious thrombocytopenia

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Can be induced by antiplatelet antibodies, presence of these antibodies are responsible for clearing aged and damaged platelets.

Usually IgG type, some are IgM and IgA (directed against GPIIb-IIIA)

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Antibody-induced platelet agglutination

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What are the anticoagulants that can cause platelet clumping?

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EDTA

NA CITRATE

NA OXALATE

ACD

HEPARIN

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Platelets forms a rosette around the periphery of leukocytes.

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Platelet satellitism

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Most commonly cell involved in Platelet satellitism.

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Neutrophils

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Some antiplatelet antibodies from patient with pseudothrombocytopenia cross react with negatively charged phospholipids and may exhibit cardiolipin activity.

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Antiphospholipid Antibodies

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Treatment for patients suffering from acute coronary syndromes.

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GP IIb/IIIA Antagonists

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Has been associated with both pseudo and true thrombocytopenia.

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Abciximab

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An accurate platelet count can be obtained only by sampling blood into _____________ and manual count using ___________.

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Ammonium oxalate

Burker chamber

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Presence of severe thrombocytopenia, absence of megakaryocytes in the bone marrow.

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Congenital Amegakaryocytic Thrombocytopenia

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More severe type of thrombocytopenia with constantly low platelet count and an early onset of pancytopenia.

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Group CAMT I

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Transient increase in platelet counts during the first year of life and a later or no development of pancytopenia.

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Group CAMT II

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Rare disease first identified in 1959.

Thrombocytopenia is moderate approximately 50X10(3)/uL.

Megakaryocytes are low in number, absent or appear immature.

CAN BE MANAGED BY PLATELET TRANSFUSION.

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Thrombocytopenia Absent Radius

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Caused by mutation of MYH9 gene.

Affected patients have triad of thrombocytopenia, macrocytosis and dohle-body like inclusions.

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MYH9-related thrombocytopenia

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Characterized by abnormal binding of large vWF multimers of platelets.

Mild thrombocytopenia, increased ristocetin-induced platelet aggregation.

Resembles type 2b von Willerbrand's disease.

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Platelet-type von Willerbrand's disease

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Relatively common and mild form of macrothrombocytopenia and has been described in a group of healthy subjects for Italy and Balkan peninsula.

Gene mutation to the short arm of chromosome 17.

PBS shows platelets larger than normal.

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Mediterranean Macrothrombocytopenia

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Characterized by several congenital anomalies including dysmegakaryopoiesis.

Clinical features include mild to moderate psychomotor retardation, trigoencephaly, facial dysmorphism, cardiac defects and thrombocytopenia.

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Paris-Troussaeu Syndrome

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It is associated with bleeding tendencies and classical abnormal platelet morphology.

Alpha granules are absent or greatly reduced.

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Gray platelet Syndrome

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Known as "X-linked hereditary disorder" associated with combined immunodeficiency thrombocytopenia, small platelets, eczema and an increased risk to autoimmune disorders and cancer.

Microthrombocytopenia is the most consistent feature.

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Wiskott-Aldrich Syndrome

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Primary results from marrow failure.

Decreased proliferation and differentiation of megakaryocyte progenitor similar to aplastic anemia.

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Proxysmal Nocturnal Hemoglobinuria

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Are clonal myeloid disorders characterized by blood cytopenias in combination with a hypercellular marrow that often exhibit dysplastic changes in any of the three hematopoietic lineages.

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Myelodysplastic syndrome

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AKA "IMMUNE THROMBOCYTOPENIA"

One of the most common disorders causing severe isolated thrombocytopenia.

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Idiopathic Thrombocytopenic Purpura

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First described as a pentad of signs and symptoms that include: thrombocytopenia, microangiopathic hemolytic anemia, fever, neurologic abnormalities and renal dysfunction.

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Thrombotic Thrombocytopenic Purpura

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Are the characteristic pathologic feature and are found in multiple organs.

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Hyaline microthrombi

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Associated with ADAMTS13 deficiency.

Blood smear showing polychromasia, basophilic stippling, nucleated cells, schistocytes.

Platelets counts <20X10(9)/L

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Acquired Thrombotic Thrombocytopenic Purpura

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AKA SHULMAN-UPSHAW SYNDROME OR CHRONIC RELAPSING TTP.

Hemolysis with schistocytes on blood smears may be noted.

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Hereditary Thrombotic Thrombocytopenic Purpura

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Syndrome of small vessel renal thrombi, microangiopathic hemolysis that occurs following a prodrome of hemorrhagic diarrhea.

Characterized clinically by microangipathic hemolytic anemia thrombocytopenia and renal failure.

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Hemolytic Uremic Syndrome

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More commonly seen in pediatric population.

Pathologic thrombi are almost always limited to the glomerular capillaries and afferent arterioles of the kidney.

Infection with Enterohemorrhagic E. coli 0157:H7.

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Hemolytic Uremic Syndrome

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Infection with Enterohemorrhagic E. coli 0104:H4 and S. dysenteriae serotype 1 have been implicated as causative agents.

Appearance of dark-red or nearly black urine that may quickly lead to oliguria or even anuria.

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Shiga Toxin associated Hemolytic Uremic Syndrome

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Caused by many illness, sepsis, obstetric emergencies and severe trauma, and may cause bleeding.

Thrombocytopenia is usually seen.

Appears to be accelerated platelet destruction in combination with coagulation factors consumption.

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Disseminated Intravascular Coagulation

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Low level of ADAMTS13 is detected.

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Pregnancy Associated Thrombocytopenia

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Defined by hypertension and proteinuria; usually become evident during second trimester and is a major contributor to maternal and fetal morbidity and mortality.

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Preeclampsia

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Defined by the occurence of acute neurologic abnormalities in a preeclamptic women during peripartum period.

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Eclampsia

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Disorder related to preeclampsia/eclampsia and is seen in the peripartum period and defined by the presence of microangiopathic HA, elevated liver enzymes and low platelet count.

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HELLPS Syndrom