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Kriti Baba Shrestha Roll no: 23 5 th Batch, 3 rd Year Department of Oral Pathology.

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Presentation on theme: "Kriti Baba Shrestha Roll no: 23 5 th Batch, 3 rd Year Department of Oral Pathology."— Presentation transcript:

1 Kriti Baba Shrestha Roll no: 23 5 th Batch, 3 rd Year Department of Oral Pathology.

2  Purpura Nonthrombocytopenic Purpura Thrombocytopenic Purpura  Primary Thrombocytopenia  Thrombotic Thrombocytopenic Purpura  Wiskott-Aldrich Syndrome  Thrombocytasthenia Familial Thrombasthenia Thrombocytopathic Purpura Thrombocythemia

3 Purpura is defined as a purplish discoloration of the skin and mucous membranes due to the spontaneous extravasation of the blood. It is a symptom rather than a disease entity. Purpura mainly results if the blood platelets are defective or deficient, as the clotting mechanism is the important function of blood platelets. Classification:- 1) Nonthrombocytopenic Purpura 2) Thrombocytopenic Purpura a. Primary or ‘essential’ purpura b. Secondary or symptomatic purpura

4 Fig: Oral Purpura Fig: Purpura on lower extremities

5  It is the type of purpura which is not mediated through changes in the blood platelets, but rather through alteration in the capillaries themselves that results in many instances in increased permeability.  The most common cases or conditions causing this form of purpura are: I. Autoimmune a.Allergic purpuras b.Drug-induced vascular purpura c.Purpura fulminans  II. Infections a. Bacterial (meningococcemia and septicemia due to other organisms, typhoid fever, scarlet fever, diphtheria etc. b. Viral (small pox, influenza, measles, others)  III. Structural malfunctions a. Hereditary hemorrhagic telangiectasia b. Hereditary disorders of connective tissue ( osteogenesis imperfecta, Ehlers-Danlos syndrome etc.) c. Acquired disorders of connective tissue (scurvy, Cushing's disease, senile purpura)

6  IV. Miscellaneous a.Autoerythrocyte sensitization and related syndromes (DNA hypersensitivity cutaneous hyper-reactivity of hemoglobin) b.Paraproteinemias (hyperglobunemic purpura, Waldenstrom’s purpura) c.Purpura simplex and related disorders d.Purpura in association with certain skin disease( ‘orthostatic’ and ‘mechanical’ purpura)


8  It is a disease in which there is an abnormal reduction in the number of circulating blood platelets.  Development of the focal hemorrhages into various tissues and organs, including the skin and mucous membranes.  There are two basic forms of thrombocytopenia : a.Primary (of unknown etiology) b.Secondary (which may be due to various situations)


10 Fig: Secondary thrombocytopenia

11  Primary thrombocytopenia is thought to be an autoimmune disorder in which a person becomes immunized and develops antibodies against his/her own platelets.  In some cases in it appears to be absence of a platelet- stimulating or megakaryocyte-ripening factor.  The various manifestations of Primary and Secondary thrombocytopenic purpura are near Identical so may be described together.

12  Age : Primary thrombocytopenia occurs before 3 rd decade of life, greatest incidence before 1 st decade. Secondary thrombocytopenia has no particular age predilection.  Sex: Female> Male (especially women of child bearing age)  Characterized by the spontaneous appearance of purpuric or hemorrhagic lesions of the skin of varying size from tiny, red pinpoint petchiae to large purplish ecchymoses.  Bruising tendency  Epistaxis, hematuria, gastrointestinal bleeding, producing melana.  Hemiplegia

13  Severe and profuse gingival hemorrhage (prominent) which may be spontaneous and often arise in the absence of skin lesion.  Petchiae also occur on oral mucosa, commonly at palate. appear as numerous tiny, grouped clusters of reddish spots of a millimeter or less in diameter.  Actual ecchymoses do occur occasionally.  Tendency of excessive bleeding.



16  Platelet count is usually >60,000 per cubic millimeter.  Bleeding time is prolonged, up to 1 hour or more.  Capillary fragility is increased.  Positive Tourniquet test.  Red and white blood cells normal unless secondarily affected by frequent episode of hemorrhage or drugs.  Normal coagulation time.  Giant platelets may be seen in peripheral smear which may suggest congenital thrombocytopenia.

17  No specific treatment.  Splenectomy proved to be beneficial.  Corticosteroids used in many cases with excellent results though recession may be temporary.  Prognosis for the patients are fairly good, since recession is common.  In the secondary thrombocytopenia, correction or the removal of the etiologic factors is essential.

18  It is a life threatening multisystem disorder of an obscure nature but may be immunologically mediated.  1 st described by Eli Moschcowitz in  It is characterized by microangiopathic hemolysis and platelet aggregation/hyaline thrombi in micro-circulation, whose formation is unrelated to coagulation activity.  The endothelia of kidney and brain are particularly vulnerable to TTP.

19  Age: usually in young adults.  Sex: female>male. (associated with pregnancy, HIV, cancer, bacterial infections, bone marrow transplantation etc.)  Characterized by thrombocytopenia, hemolytic anemia, fever, transitory neurologic dysfunction and renal failure. Histologic Features  Widespread microthrombi in the arterioles, venules, and capillaries throughout body.  Intravascular thrombi composed of loose aggregates of platelets organized into amorphous plugs replaced by fibrin.  Characteristic microscopic gingival changes as occlusive subintimal deposits of PAS(periodic acid-Schiff) positive material at arteriolocapillary junction.

20 Fig: thrombotic thrombocytopenic purpura

21  On blood examination thrombocytopenia and anemia noted.  Fragmented RBCs consistent with hemolysis noticed in peripheral smear.  Reticulocyte count elevated.  Prothrombin time and activated partial thromboplastin time (aPTT) are in normal limits.  LDH levels increased.  Indirect bilirubin elevated (due to extensive hemolysis).  Urinalysis shows protienuria and microscopic hematuria). Treatment and Prognosis  Disease uniformly fatal  Survival supported by modern therapeutic drugs and techniques including corticosteroids, platelets, aggregation inhibitors, Splenectomy and exchange transfusions.

22  Wiskott-Aldrich syndrome (WAS) is an X-linked recessive genetic condition with variable, commonly includes immunoglobulin M (IgM) deficiency.  This syndrome results from an X-linked genetic defect in a protein now termed as Wiskott-Aldrich Syndrome protein (WASp) whose function seems to bridge between signaling and actin polymerization in the cytoplasm.

23  Usually seen exclusively in young boys.  Characterized by thrombocytopenic purpura, eczema, usually beginning from the face.  Petechiae and a purpuric rash or ecchymoses of the skin may be the early sign.  Manifest boils, otitis media, bloody diarrhea, respiratory infection.  Occurrence of a lymphoreticular malignant neoplasm, commonly a malignant lymphoma.  Increased susceptibility to infection appears related to an antibody deficiency.

24  Spontaneous bleeding of the gingiva (most frequently seen).  Gastrointestinal bleeding and Epistaxis also can be seen.  Palatal Petechiae may also be present.  Laboratory Findings  Both qualitative and quantitative abnormality of platelets.  Prolonged bleeding time (due to thrombocytopenia between 18,000-80,000 / cubic millimeter)  Alteration in size and shape of the platelets. (smaller)  Quantitatively, decreased production and defective maturation of platelets since normal megakaryocyte seen in marrow.  Accelerated platelet clearance from peripheral blood.


26  No specific treatment for the disease. Death usually occurs within the first 5 years of life as a result of secondary infection or hemorrhage.  Treatment with some antibiotics and platelet transfusion.  Bone marrow transplantation.  Transfer factor.  The eventual prognosis, however, is poor.

27  Thrombocysthenia is the term used to designate a variety of diseases characterized by a qualitative defect in blood platelets.  Some forms are congenital and/or familial, while others are acquired. A. Familial Thrombasthenia B. Thrombocytopathic Purpura C. Thrombocythemia

28  It is a hereditary, chronic hemorrhagic disease transmitted as an autosomal recessive trait.  There appears to be at least 7 forms of Glanzmann disease, thus accounting for the heterogeneous nature of the various description of the condition. Clinical Features  No sex predilection, though in female onset of menarche may be critical event.  Exhibit usual characteristics of excessive bleeding either spontaneous or due to minor trauma.  Purpuric hemorrhage of skin, epistaxis, gastrointestinal bleeding  Hemarthrosis also been reported.

29 Fig: familial Thrombasthenia

30  Spontaneous bleeding from the oral cavity, particularly gingival bleeding.  Often seen in these patients as are palatal petechiae.  Laboratory Findings  Prolonged bleeding time  Clot retraction characteristically impaired.  Platelet count and clotting time are normal.  Reduced amounts of certain membrane glycoprotein on the surface of platelets which is responsible for haemostatic defect.


32  No specific treatment.  However, Perking and his co-workers discussed this disease and reported two cases of patients requiring oral surgery who were treated with microfibrillar collagen preparation and with a fibrinolytic inhibitor, c- aminocaproic acid, to control post operative hemorrhage.

33  It is a group of rare diseases of unknown etiology in which the patient manifests a bleeding tendency referable to qualitative defects in the blood platelets.  Not related to thrombocytopenia as the platelet count is normal, though these two diseases are clinically indistinguishable.

34  Severe bleeding tendency and bruise easily after only minor trauma.  Spontaneous ecchymoses (common)  Petechial hemorrhage (rare).  Epistaxis and bleeding into gastrointestinal tract are frequent clinical findings.  In females, sometimes severe menstrual bleeding occurs which may require blood transfusion.

35  Spontaneous gingival bleeding (most common).  Mucosal ecchymoses (occasional).  Excessive and prolonged bleeding from dental extractions. Can be serious management plan. Laboratory Findings  Bleeding time may be normal or prolonged.  Platelet counts nearly normal.  Failure of normal aggregation.  Variety of platelet defect seen within its different forms for eg: in ‘storage pool disease’, there is deficiency in nonmetabolic storage pool of platelet adenine nucleotides.


37  No satisfactory treatment for the disease.  Conventional haemostatic agents and blood transfusion aid in controlling the severe hemorrhage.  Death due to prolonged bleeding is rare but could obviously occur.

38  Thrombocythemia is a condition characterized by an increase in the number of circulating blood platelets.  A number of cases have been reported to occur in association with the polycythemia and myeloid leukemia, anemia, tuberculosis and sarcoidosis, rheumatoid arthritis, bronchial carcinoma with osseous metastases.  As in the thrombocytopenia, 2 forms are recognized:- a.Primary or Essential thrombocythemia a.Secondary thrombocythemia

39  Primary thrombocythemia is of unknown etiology.  Secondary thrombocythemia may occur after, Traumatic injury Inflammatory conditions Surgical procedures Parturition  It may be due to over production of the proinflammatory cytokines, such as IL-1, IL-22, that occurs in inflammatory, infections, malignant stages.  These cytokines may be involved in relative thrombocytosis.

40  No age or gender predilection.  Asymptomatic in some cases.  Invariably shows the bleeding tendency though the blood platelet count is elevated.  Epistaxis and bleeding into gastrointestinal tract as well in genitourinary tract and Central Nervous System are common.  In skin: hemorrhage are seen.  Oral Manifestations  Spontaneous gingival bleeding (common), petechiae (rare).  Excessive and prolonged gingival bleeding after dental extractions.  Oral hemorrhage.

41  Markedly increased platelet count. ◦ Interferes with the formation of the thromboplastin.  Abnormal platelets aggregation in response to several aggregating agents.  Normal:-.Clotting time.Prothrombin time.Clot retraction.Tourniquet test.  Bleeding time prolonged.  In Primary thrombocythemia,RBC and WBC count normal and are altered in Secondary thrombocythemia depending the associated condition.

42  Administration of Radioactive Phosphorous {P32} (most common).  Blood transfusion in case of severe hemorrhage.  Certain cytotoxic drugs, Heparin during thrombotic episodes, Corticosteroids and Aspirin have been used with some degree of success.



45  Shafer’s Textbook of Oral Pathology  Oral And Maxillofacial Pathology, Neville.  Internet sources.

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