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Platelet related disorders and syndromes
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Diseases Involving Blood Platelets
By Suparn Kelkar Roll no 25 3rd 2nd
Functions of Platelets
Primary Arrest of bleeding Participate in blood clotting mechanism
Overview of Diseases involving Platelets Purpura1) Nonthrombocytopenic purpura2) Thrombocytopenic purpurai. Primaryii. Secondary Thrombotic Thrombocytopenic Purpura Wiskott-Aldrich Syndrome Familial Thrombasthenia Thrombocytopathic Purpura Thrombocythemia
Purpura
It is defined as a purplish discoloration of the skin and mucous membrane due to spontaneous extravasation of blood and is itself a symptom rather than a disease entity
ThrombocytastheniaIt is the term used to designate a variety of diseases characterized by a Qualitative defect in blood
Nonthrombocytopenic PurpuraPurpura due to alteration in capillariesCausesA. AutoimmuneB. InfectionsC. Structural Malformations
D Miscellaneous
Oral ManifestationsResemble thrombocytopenic purpura
Thrombocytopenic PurpuraThis is a disease in which there is a abnormal
reduction in the NUMBER of circulating Platelets
Causes focal hemmorhage into various tissues and organs including skin and mucous membrane
2 typesi. Primary or essentialii. Secondary or symptomatic purpura
Primary Thrombocytopenia
Autoimmune disorderAntibodies are produced againsts patients
own plateletsCauses decrease in platelet count due to:1. Antiplatelet Globulin2. Absence of platelet stimulating/
megakaryocyte ripening factor Acute form-----affects children Chronic form----affects mostly female and
adults
Secondary ThrombocytopeniaEtiologyI. Conditions associated with REDUCTION
of platelet count
Infiltration of Marrow by abnormal cellsMegaloblastic Anemia Metabolic disorders
InfectionBacterial :Spirochetal :RickettsialViral :
II.Conditions associated with a reduction in LIFESPAN of Platelets
Diseases Related to Mechanism
Diseases Resulting in platelet utilization at an increased rate
III. Thrombocytopenia due to DILUTION of platelet by transfusion of platelet poor blood
IV. Conditions in which thrombocytopenia is of IDIOPATHIC PATHOGENESIS
Clinical Features1) Age: 10-30 yrs, no sexual predilection 2) Characteristics Spontaneous appearance of purpuric or
hemorrhagic lesions of skin These vary in size From tiny red pinpoint petechiae to large
purplish ecchymoses to massive hematoma
Pt feels burning sensation in these areasEpistaxis i.e. bleeding from nose, urinary
tract bleeding causing hematuria , bleeding in GIT causing melenema or hematemesi
Spleen is not palpable***If palpable the it is due to LEUKEMIA
Oral Manifestations1. Severe or Often Profuse GINGIVAL
hemmorhage
Petechiae on oral mucosa , commonly on palate seen as tiny grouped clustures 1mm or less in diameter
Sometimes Ecchymoses occur
Laboratory findingsPlatelet count : below 60,000 per cu mmBleeding time : 1hr or moreClotting time : normalRBC and WBC count : normalIf giant platelets present on peripheral blood
smear then ---- it is CONGENITAL thrombocytopenia
Treatment and PrognosisNo specific treatmentSplenectomy can be doneFor symptomatic releif -- blood transfusion and bed restDrugs : Corticosteroids therapy bt may relapse In secondary thrombocytopenia: correction or removal of etiologic factors is essential Death occurs due to sudden severe hemorrhagePrognosis is GOOD
Thrombotic Thrombocytopenic Purpura (TTP)Also known as Moschcowitz diseaseUncommon form of Thrombocytopenic
purpuraIt is a life threatening multisystem disorder of
an obscure nature but may be immunologically mediated
Described by Eli Moschowitz in 1924
EitiologyHIV ,Cancer ,Bacterial infection, Bone marrow
Transplantation and other drugs
Mechanism of ActionMicroangiopathic hemolysis and platelet
aggregration/Hyline thrombi in microcirculationThrombi -- Occlude vascular lumiaEndothelia of BRAIN and KIDNEYS are most
affected
No inflammatory response is seenBut fragments of erythrocytes and hemolysis
is seen in blood
CLINICAL FEATURES1. Age : Young Adults2. Sex : Females more commonly affected3. Thrombocytopenia4. Hemolytic anemia5. Fever6. Transitory neurologic dysfunction7. Renal failure
Histologic FeaturesWidespread microthrombi in blood vessels
such as Arteriole, Venules, and Capillaries
Gingival tissue show subintemal deposits of PAS stain at Arteriocapillary junction
Laboratory Findingsdecreased platelet count, Hb is decreasedFragmented RBC’s (schistocytes)Reticulocyte count IncreasedProthrombin time : NormaLactivated platelet thromboplastin time (aPTT) :
NormalLDH levels increasedIndirect Bilirubin levels increasedUrine Examination :Proteinuria + Hematuria seen
Treatment and PrognosisBut now modern therapeutics drugs and
techniques such as
Corticosteroids, Platelet aggregation inhibitors, Splenectomy, And exchange transfusions provide treatment options
Wiskott-Aldrich SyndromeAlso known as Hypogammaglbinemia MThis is a X-linked recessive genetic condition
with variable expression, commonly include immunoglobulin M (IgM) deficiency
Severe congenital immunodeficiency
Clinical FeaturesSex : Exclusive to MalesCharacterized by thrombocytopenia purpura,
eczema on face
Petechiae and purplish rash or ecchymoses of the skin
Eczema is allergic in nature Pt commonly manifests1. Boils2. Otitis Media3. Bloody diarrhea4. And respiratory infection Imp Features1. Occurance of LYMPHOETICULAR MALIGNANT
NEPLASIAS2. Malignant lymphoma (most common)
ORAl manifestationsSpontaneous gingival bleeding and palatal
petechiae seen
Laboratory FindingsThrombocyte count : 18,000-80,000 per cu
mm of bloodBleeding time : IncreasedAnisocytosis i.e. change in SIZE and SHAPE of
platelets : Platelets are usually Smaller in size
Qualitatively : Production and Maturation of immature platelets is decreased overall
Treatment and Prognosis No specific treatmentAntibiotics and Platelet transfusions, bone
marrow transplantation are available treatment options
Overall Prognosis is POORDeath occurs due to secondary infection or
severe hemorrhage within 1st 5 yrs of life
Familial ThrombastheniaCommonly known as Glanzmann
thrombasthenia or diseaseIt is a hereditary, chronic hemorrhagic disease
transmitted as an autosomal recessive trait
Clinical FeaturesSex : Both genders affectedBut in females onset of menarche can cause
this disorderPatients exhibit excessive bleeding eighter
spontaneously or following minor traumaPurpuric hemorrhages of skinEpistaxis and GIT bleedingHemarthrosis seen in some cases
Oral manifestations : spontaneous gingival bleeding, palatal petechiae
Laboratory findings1. Bleeding time : Prolonged2. Cloth retraction is impaired3. Clotting time : Normal4. Platelet count : Normal5. Reduced amounts of membrane
glygoprotiens on surface of platelet
TreatmentNo specific treatmentMicrofibrillar collagen preparations with
fibrinolytic inhibitor, c-aminocarproic acid are employed to control postoperative hemorrhage
Thrombocytopathic PurpuraAlso known as thrombocytopathiaThese are a group of rare diseases with
unnown eitiology in which patients manifest a bleeding tendancy referable to qualitative efects in blood platelets
Platelet count remains normal, therefore is different from thrombocytopenia
Clinical featuresPatients have Severe bleeding tendency and
Bruise easily after minor traumaSpontaneous Ecchymoses seenEpistaxis and bleeding into GIT are a common
findingIn females menstrual bleeding becomes so
severe that blood transfusions may be required
Oral Manifestations1. Spontaneous gingival bleeding2. Mucosal ecchymoses
Laboratory FindingsPlatelet count : normalBleeding time : normal or prolongedBernard-Soulier syndrome : abnormal
response to fibrinogen
TreatmentNo specific treatment availablePriority of treatment is symptomatic control
i.e control of severe hemorrhageDeath is rare but has occurred in a few cases
ThrombocythemiaAlso known as thrombocytosis or increase in number
of platelet count2 forms are recognisedi. Primary (essential)ii. Secondary Eitiologyi. Primary : Unknownii. Secondary : Traumatic injury, inflammatory
responses, surgery , parturition, polycythemia , myeloid luekemia, anemia, tuberculosis, sarcoidosis, hyperadrenalism, rheumatoid arthritis, bronchial carcinoma with osseous metastases
Mechanism of ActionIn secondary thrombocytosis there is overproduction of
proinflammatory CYTOKINES such asi. IL-1ii. IL-6iii. IL-11 Along with C-reactive protein, G-CSF , GM-CSF All these factors together lead to increase in platelet
count when there is i. Chronic inflammationii. Infectioniii. Malignancies
Cinical FeaturesNo gender or age predilection is seenHemorrhage found on skinEpitaxis and bleeding into GIT and nervous
systemFew patients remain asymptomatic and are
identified on routine blood count
Oral manifestationsSpontaneous gingival bleeding is commonPetechiae are rare***Excessive and prolonged bleeding after dental
extraction
Laboratory findingsPlatelet count : greatly increased, i.e. could be
as high as 1,40,00,000 per cu mmAbnormal platelet aggregrationBleeding time: ProlongedClotting time: normalProthrombin time: normalClot Retraction :normalIn secondary thrombocythemia RBC and WBC
counts are abnormal
TreatmentAdministration of Radioactive Phosphorus
(P32)Blood transfusions in cases of severe
hemorrhageDrugs : Aspirin, Heparin (in cases of
thrombotic episodes) , Corticosteroids are administered
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