Rakta Pitta: Hematological Diseases Presentation

MEDICINE III Level V - Semester I Lesson 02 - Hematological Disease Topic 03 – Rakta pitta By Dr. Eranga Karandugoda Dept. of Cikitsa • Lab Tests • CBC-Plt • BT,(CT) • PT Neural • PTT Blood Vessel Constriction BV Injury Tissue Factor Platelet Aggregation Coagulation Cascade Primary hemostatic plug Reduced Platelet Activation Blood flow Stable Hemostatic Plug Fibrin formation The Clotting Mechanism INTRINSIC EXTRINSIC Collagen Tissue Thromboplastin XII XI VII IX VIII X V PROTHROMBIN (II) FIBRINOGEN (I THROMBIN (III) FIBRIN Laboratory evaluation • Platelet count • Bleeding time (BT) • Prothrombin time (PT) • Partial thromboplastin time (PTT) • Thrombin time (TT) Platelet count NORMAL 100,000 - 400,000 CELLS/MM3 < 100,000 Thrombocytopenia 50,000 - 100,000 < 50,000 Mild Thrombocytopenia Severe Thrombocytopenia Bleeding disorders Vascular abnormalities Platelet disorders Clotting factor abnormalities DIC Vessel Defects Causing Bleeding • Begins with bleeding episode in presence of normal lab tests for coagulation function • Types divided into hereditary and acquired • Symptoms are usually of the superficial ones. • Usually these diseases are diagnosed by exclusion. After ruling out PLT disorders, coagulation or fibrinolytic disorders in a patient who has bleeding symptoms. Vascular Diseases • PLT count and screening tests for coagulation factors are usually normal. • PLT function tests such as bleeding time and other PLT function tests are also normal, but BT may be prolonged in some vascular diseases. Inherited vascular disorders • They are very rare, and while bleeding is a common symptom, hemostasis tests are NOT necessary for diagnosis. Hereditary Connective Tissue Defects • Defect affects the ability to support vessel walls • Examples – Ehlers-Danlos Syndrome • Lack of structural tissue support (collagen disorders) • Skin elasticity and fragility. • Hypermobility of joints • Evidenced by bleeding/bruising • Recurrent joint problems & scarring of the face. • The most serious is deficiency of type III collagen (blood vessel type). Which leads to Acute & severe Internal bleeding & sudden death. • Pseudoxanthoma Elasticum – Autosomal recessive trait – Lack of skin elasticity – Some connective tissue calcified – Bleeding and bruising evident Hereditary Vessel Disease Hereditary Hemorrhagic Telangiectasia (HHT) • Inherited as an autosomal dominant trait • Defect of angiogenesis • Involves bleeding from abnormally dilated vessels “telangiectasias” • Vessels involved do not contract normally and collapse easily • Patient has pinpoint lesions (tiny areas of bleeding) • Lesions occur on the face, hands, and feet • May develop at all ages • Blood loss may cause anemia • Diagnosis based on physical appearance Kasabach-Merritt syndrome (Hemangioma) • Benign tumor of vascular tissue • Grow rapidly to a giant proportion. • Threaten the function of neighboring tissues. • Mechanical injury may result in severe bleeding. • May trigger a localized DIC with thrombocytopenia & consumption coagulopathy, thereby worsening bleeding. • Tumor composed of many blood vessels (blood-filled) HT or also called Cavernous Hemangioma • Lesion may swell and bleed • Tumor site may form clots, hemolyzed RBCs and vessel obstruction • Present at birth Acquired Connective Tissue Defects Vitamin C Deficiency (Scurvy) – Caused deficient Vitamin C – Vitamin C required for vessel collagen integrity – Acts as “cement” holding endothelial cells together – Lack of Vitamin C prevents proper collagen formation – Result: bleed and vessel fragility – Symptoms include gum bleeding, petechiae and bleeding into tissues and muscles – Treated with Vitamin C Senile Purpura – Occurs in elderly population – Usually benign – Collagen degradation/loss affects vessel integrity – Bruising on arms/hands – No treatment/therapy available Other Vascular Disorders Self-produced “autoantibodies” damage to vessels – Caused by drugs resulting in purpura – Caused by allergic/immune disturbance Evidenced by swelling, ulcers, purpura and lesions and other symptoms • Affects children • – Other allergic purpura-Hemoch-Schonlein variety Accompanied by joint and abdominal pain • Avoidance of allergen aids recovery • Infectious purpura – Observe petechiae and purpura – Results from  Inflammatory response to agent  Autoimmune/autoantibody response  Bacterial products or toxins  Injury caused by agent – Low platelets observed and DIC – Cure is to treat infection Platelet Disorders Classification: Quantitative PLT Disorders Thrombocytopenia Thrombocytosis PLT Reference Range = 150 - 450 x109/L Thrombocytopathy Quantitative PLT Disorders Acquired Coagulation Disorders • Vitamin K deficiency • Warfarin therapy • Liver disease • Antibodies against factor VIII (or other factors) • Massive transfusion • Thrombocytopenia – False reading • Clumping of platelets • Platelet satelitism – Margination in congestive splenomegaly – True thrombocytopenia • Failure to produce platelets – Acquired - drugs - alcohol – Inherited – ,May-Hegglin. Wiscott-Aldrich • Platelet destruction – Immune • Idiopathic • Disorders of platelet function – Inherited • Von Willebrand’s disease • Glanzmann’s • Bernard-Soulier • Storage pool disease – gray platelets – Acquired • Drugs • Multiple myeloma – hyperglobulinemia • Uremia Coagulation disorders • Hemophilias A & B • Others • Von Willebrand’s disease Thrombocytopenia • Reduced platelet count. • The most common cause of excess or abnormal bleeding. • thrombocytopenia is also a symptom of a disease Platelet count Symptoms • 50-100 X109/L Prolonged bleeding following trauma • < 50X109/L Easy bruising Purpura following minor trauma • < 20 X109/L Spontaneous bleeding Petechiae May suffer spontaneous internal and intracranial bleeding Causes Thrombocytopenia • Usually mucosal bleeding • Epistaxis, menorrhagia, and GI bleeding is common • Trauma does not usually cause bleeding Thrombocytopenia • Three mechanisms of Thrombocytopenia – Decreased production • Usually chemotherapy, myelophthisic disease, or BM effects of alcohol or thiazides – Splenic Sequestration • Rare • Results from malignancy, portal hypertension, or increased Splenic RBC destruction ( hereditary spherocytosis, autoimmune hemolytic anemia) – Increased Destruction Thrombocytopenia • Immune thrombocytopenia – Multiple causes including drugs, lymphoma, leukemia, collagen vascular disease – Drugs Include • Digitoxin, sulfonamindes, phenytoin, heparin, cocaine, Quinine, quinidine, glycoprotein antagonists – After stopping drugs platelet counts usually improve over 3 to 7 days – Prednisolone (1mg/kg) with rapid taper can shorten course ITP • Diagnosis of exclusion • Associated with IgG anti-platelet antibody • Platelet count falls to less that 20,000 Acute Form – Most common in children 2 to 6 years – Viral Prodrome common in the 3 weeks prior – Self Limited and > 90% remission rate – Supportive Treatment – Steroids are not helpful ITP • Chronic Form – Adult disease primarily – Women more often than men – Insidious onset with no prodrome – Symptoms include: easy bruising, prolonged menses, mucosal bleeding – Bleeding complications are unpredictable – Spontaneous remission is rare Inherited disorders of platelet function • Defects in platelet – Vessel Wall interactions Von Willebrand disease • Defects in platelet – platelet interactions Congenital afibrinogenemia • Defects in platelet Granules, Secretion and Signal Transduction • Defects of platelet procoagulant function • Defects in structural companents Hemophilia • Hemophilia is an inherited bleeding disorder in which there is a deficiency or lack of factor VIII (hemophilia A) or factor IX (hemophilia B) Hemophilia A and B Hemophilia • Hemophilia A and B are X-linked recessive disorders • Hemophilia is typically expressed in males and carried by females • Severity level is consistent between family members • ~30 % of cases of hemophilia are new mutations • Hemophilia A, also called factor VIII (FVIII) deficiency or classic hemophilia • Hemophilia B, also called factor IX (FIX) deficiency or Christmas disease • Factor XII , IX, a clotting protein Other Factor Deficiencies • Factor deficiencies such as I, II, V, VII, X, XI, XII and XIII, rare disorders. These very rare factor deficiencies Detection of Hemophilia • Family history • Symptoms – Bruising – Bleeding with circumcision – Muscle, joint, or soft tissue bleeding • Hemostatic challenges – Surgery – Dental work – Trauma, accidents • Laboratory testing Screening Tests • PT is prolonged or (test extrinsic pathway) • Platelets are normal in count and function. • TT (thrombin time): prolonged in disorders of fibrinogen. • If any test is abnormal of these screening tests, additional testing may resolve the disorder Additional Testing • Specific Factor Assays. • Fibrinogen Level • Antithrombin Level von Willebrand’s Disease • Family of bleeding disorders • Caused by a deficiency or an abnormality of von Willebrand Factor • Von Willebrand disease (VWD) is a genetic disorder caused by missing or defective von Willebrand factor (VWF) • A clotting protein. VWF binds factor VIII, a key clotting protein, and platelets in blood vessel walls • Which help form a platelet plug during the clotting process. The condition is named after Finnish physician Erik von Willebrand vWF Production • Vascular endothelial cells • Megakaryocytes • Most vWF is secreted • Some vWF is stored – Weibel-Palade bodies in endothelial cells – Alpha granules of platelets • Constitutive and stimulus-induced pathways • Release stimuli – Thrombin – Histamine – Fibrin • Release stimuli (platelets) – Thrombin – Collagen • Carrier protein for Factor VIII (FVIII) – Protects FVIII from proteolytic degradation – Localizes FVIII to the site of vascular injury – Hemophilia A: absence of FVIII Clinical Manifestations • Most with the disease have few or no symptoms • For most with symptoms, it is a mild manageable bleeding disorder with clinically severe hemorrhage only with trauma or surgery • Types II and III: Bleeding episodes may be severe and potentially life threatening • Disease may be more pronounced in females because of menorrhagia • Bleeding often exacerbated by the ingestion of aspirin • Severity of symptoms tends to decrease with age due to increasing amounts of vWF Symptoms • Frequent nosebleeds • Easy bruising • Excessive bleeding during and after invasive procedures (such as tooth extractions and surgery ) • Women often experience menorrhagia heavy menstrual periods Disseminated Intravascular Coagulation • An acquired syndrome characterized by systemic intravascular coagulation • Coagulation is always the initial event • Most morbidity and mortality depends on extent of intravascular thrombosis • Multiple causes Pathophysiology of DIC • Activation of Blood Coagulation • Suppression of Physiologic Anticoagulant Pathways • Impaired Fibrinolysis • Cytokines Rakta pitta nirukti s<yaegadœ Ë;[at! tÄu samaNyadI gNXv{yaeR> rktSy ipÄmaOyat< rktipÄ< mini;ÉI> • s<yaegadœ Ë;[at! tÄu - • samaNyadI gNXv{yaeR> - • r´Sy ipÄmaOyat< - • rípÄ< miti;ÉI> - Rakta pitta Nidana & Samprapti "mRVyayam zaekaXv VyvayEritseivtE> tIú[ae:[ ]ar lvnE AMlE> kquiÉrevc ipÄ< ivdGx< Svgu[Erœ ivdahTyazu zaei[tm! tw> àvtRte r´ ^Xv¡ c Axae iÖxaip va ^Xv¡ nasai] k[aRSyErœ mef+yaeingudErx> k…ipt< raemkªpEí smStEs! tt! àvtRte Rakta pitta nidana and samprapti • "mR • Vyayam - • zaekaXv - • VyvayEritseivtE> • tIú[ae:[ ]ar lvnE AMlE> kquiÉrevc • ipÄ< ivdGx< Svgu[Erœ - Rakta pitta nidana and samprapti • ivdahTyazu zaei[tm! • tw> àvtRte - - • r´kt ^Xv¡ c Axae iÖxaip va • ^Xv¡ nasai] k[aRSyErœ - • mef+yaeingudErx> • k…ipt< raemkªpEí • smStEs! tt! àvtRte - Rakta Pitta Purvarupa Sdn< zItkamITv< k{QœxUmayn< vim laeh gNxí in>ñasae rktipÄ ÉivZyit > • Sdn< - • zItkamITv< - • k{QœxUmayn< - • vim - • laeh gNxí - • in>ñasae - • rktipÄ - • ÉivZyit > - Doshabhedanugata lakshana saNÔ< s pa{fu sõeh< ipi½l< c k)aiNvtm! Zyavaé[< s)en< c tnuê]< c vaitk< rktipÄ< k;ayaÉ< k«:[< gaemuÇ siÚÉ< meckagarxUmaÉ m<jnaÉ< c pEiÄk< s<s&òil<g< s<sgaRt! iÇil<gm! siÚpatk< Doshabhedanugata lakshana • saNÔ< • s pa{fu • sõeh< • ipi½l< c • k)aiNvtm! • Zyavaé[< • s)en< c • tnuê]< c • vaitk< - Doshabhedanugata lakshana • rktipÄ< k;ayaÉ< • k«:[< - • gaemuÇ siÚÉ< - • meckagarxUmaÉ - • m<jnaÉ< c - • pEiÄk< - • s<s&òil<g< s<sgaRt! • iÇil<gm! siÚpatk<- - Sansarga bhedaya – Marga bhedaya ^Xv¡g< k)s<s&ò mxaeg< pvnanug< ÖImag¡ k)vata_y< muÉa_y<mnuvtRte Sansarga bhedaya – Marga bhedaya • ^Xv¡g< - • k)s<s&ò - • mxaeg< - • pvnanug< - • ÖImag¡ k)vata_y< - • muÉa_y<mnuvtRte - Complications - Upadrava daEbRLy ñaskas Jvr vmtu md> pa{futa dah mUDaR Éukté "aerae ivdahs! Tvx&itrip sda ù*tuLya c pIfa t&:[a kaeòSy Éed> izris c tpn< pUitinòIvnTv< Ékt Öe;a ivpakaE ivk«itrip Évedœ rktipÄaepsgR> Upadrava • daEbRLy • ñaskas • Jvr • Vmt u • md> • pa{futa • dah mUDaR • Éukté "aerae ivdahs! • Tvx&itrip sda • ù*tuLya c pIfa - • t&:[a • kaeòSy Éed> • izris c tpn< • pUitinòIvnTv< • Ékt Öe;a • AivpakaE • ivk«itrip • Évedœ rktipÄaepsgR> -

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