Purpura Disease

Allied Guru

Purpura Disease

  • Purpura ek condition hai jisme skin ya mucous membranes (jaise mouth, nose, etc.) pe purplish ya red spots dikhte hain, jo ki blood vessels ke tootne se hoti hain.
  • Jab capillaries (small blood vessels) toot jaati hain, to blood surrounding tissue mein leak ho jata hai, jisse purpura ka appearance hota hai.
  • Purpura ka size chhote petechiae (pinpoint red ya purple spots) se lekar bade bruises (ecchymosis) tak ho sakta hai.
  • Ye purpura kisi underlying condition ka sign hota hai, na ki ek alag disease.

Purpura Ke Types

Purpura ko do main categories mein classify kiya jata hai, jo platelet count aur underlying cause par depend karti hai:

1. Thrombocytopenic Purpura (Low Platelet Count)

Is type mein, platelet count bahut kam hota hai, jiski wajah se clotting ka process disturb ho jata hai aur bleeding ka risk badh jata hai. Thrombocytopenic purpura kai alag conditions se ho sakta hai, jaise autoimmune diseases, clotting factor deficiencies, aur kuch drugs ke reactions.

Key Forms:

  • Immune Thrombocytopenic Purpura (ITP):

    • ITP ek autoimmune disorder hai jisme immune system apne hi platelets ko destroy kar leta hai. Isse purpura hota hai.

    • Acute ITP zyadatar bachon mein hota hai aur viral infections (jaise chickenpox) ke baad hota hai.

    • Chronic ITP adults mein hota hai aur kai mahine ya saal tak chal sakta hai.

    • ITP ke symptoms hain: easy bruising, petechiae (chhote red spots), nosebleeds, gum bleeding, aur spontaneous bleeding.

  • Thrombotic Thrombocytopenic Purpura (TTP):

    • TTP ek rare aur severe condition hai jisme small blood clots blood vessels mein ban jaate hain, jo platelets ko consume kar lete hain.

    • Ye condition ADAMTS13 enzyme ki kami ki wajah se hoti hai.

    • TTP mein purpura ke alawa hemolytic anemia, kidney failure, neurological symptoms (confusion, seizures), aur fever hota hai.

    • Ye medical emergency hoti hai aur plasma exchange (plasmapheresis) treatment zaroori hoti hai.

  • Heparin-Induced Thrombocytopenia (HIT):

    • Ye condition heparin (ek anticoagulant) ke use se hoti hai. Isme antibodies platelets ke against ban jaati hain, jisse platelets activate ho jaate hain aur clotting hoti hai.

    • HIT mein purpura injection sites par aur dusre thrombosis symptoms ho sakte hain.

2. Non-Thrombocytopenic Purpura (Normal Platelet Count)

Is form mein platelet count normal hota hai, lekin purpura blood vessels ke weak hone, clotting factor ki kami, ya inflammation ke wajah se hota hai.

Key Forms:

  • Vasculitis:

    • Vasculitis ek condition hai jisme blood vessels inflame ho jaate hain aur unki walls weak ho jaati hain, jiski wajah se purpura ho sakta hai.

    • Common vasculitides jo purpura cause karti hain:

      • Henoch-Schönlein Purpura (HSP): Ye ek small vessel vasculitis hai, jo bachon ko hoti hai aur upper respiratory infections ke baad hoti hai. Isme purpura legs aur buttocks pe hota hai, abdominal pain, joint pain, aur kidney involvement (glomerulonephritis) ho sakta hai.

      • Polyarteritis Nodosa (PAN): Ye medium-sized arteries ko affect karta hai aur purpura ke saath abdominal pain, muscle weakness, aur organ failure ho sakta hai.

  • Scurvy:

    • Scurvy vitamin C ki deficiency ke wajah se hota hai, jis se collagen ka production disturb ho jata hai aur blood vessels weak ho jaati hain. Isse purpura, bruising, gum bleeding aur joint pain hota hai.

    • Treatment mein vitamin C supplementation di jaati hai.

  • Drug-Induced Purpura:

    • Kuch drugs purpura cause kar sakte hain, jaise:

      • Corticosteroids: Long-term use se blood vessels weak ho sakti hain.

      • Anticoagulants: Jaise warfarin, jo clotting ko inhibit karte hain aur bleeding ko badhate hain.

      • Chemotherapy drugs: Yeh bone marrow ko suppress karte hain, jisse platelets kam banne lagte hain.

  • Leukemia:

    • Leukemia ek blood cancer hai jisme bone marrow platelets produce nahi kar pata ya abnormal white blood cells blood vessels ko affect karte hain, jisse purpura hota hai.

Purpura Ke Symptoms

Purpura ka main symptom purplish ya red spots ka appearance hai skin par. Symptoms cause ke hisaab se vary karte hain:

  • Petechiae: Chhote, pinpoint red ya purple spots jo pressure dene par nahi hilte.

  • Ecchymosis: Bade bruises, jo purplish-blue ho sakte hain.

  • Purpuric Rash: Bada aur irregular rash, jo zyadatar legs pe hota hai.

  • Spontaneous Bleeding: Gum se bleeding, nosebleeds, ya blood in stool ya urine.

  • Fatigue: Anemia ya severe inflammation ke saath ho sakta hai.

  • Pain aur Swelling: HSP ya PAN mein purpura ke saath joint pain aur swelling ho sakti hai.

Lab. Diagnosis of Purpura

Purpura ka diagnosis uske underlying cause ko samajhne ke liye lab tests ki zaroorat hoti hai. Kuch common tests:

1. Complete Blood Count (CBC)

  • Platelet Count: Platelet count low ho toh thrombocytopenic purpura (ITP, TTP) ka indication hota hai, aur agar normal ho toh non-thrombocytopenic causes ho sakte hain (jaise vasculitis, scurvy).

  • Hemoglobin aur Hematocrit: Anemia ko check karne ke liye, jo bleeding ya hemolysis ke wajah se ho sakta hai.

  • White Blood Cell Count (WBC): Agar WBC elevated ho, to infection ya inflammation ho sakta hai.

2. Peripheral Blood Smear

  • Blood smear se platelets ki size aur abnormal cells ka pata chalta hai:

    • ITP mein large platelets dikhte hain.

    • TTP mein fragmented red blood cells (schistocytes) dikhenge.

    • Leukemia mein abnormal white blood cells ho sakte hain.

3. Coagulation Studies

  • Prothrombin Time (PT) aur Activated Partial Thromboplastin Time (aPTT) clotting ability ko check karte hain. Agar yeh prolonged ho, to clotting factor ki kami ho sakti hai (DIC ya liver disease).

  • D-dimer: Agar DIC ho, to D-dimer elevated hota hai.

4. Bone Marrow Biopsy

  • Agar bone marrow failure (jaise leukemia ya aplastic anemia) ka doubt ho, to bone marrow biopsy karna zaroori ho sakta hai.

5. Autoimmune Tests

  • Antiplatelet Antibodies: ITP mein antibodies platelets ke against ban jaati hain.

  • Antinuclear Antibody (ANA): Agar lupus ya koi autoimmune disease ho, to ANA levels elevated ho sakte hain.

6. Specific Tests for TTP

  • ADAMTS13 Activity: TTP mein ADAMTS13 enzyme ki kami hoti hai, jo diagnostic marker hai.

7. Infection Workup

  • Blood, urine, ya dusre cultures se infections identify kiye jaate hain, jaise meningococcal infection jo purpura cause kar sakta hai.

Treatment and Management 

Purpura ka treatment uske underlying cause par depend karta hai. Main goal hota hai bleeding ko manage karna aur root cause ka treatment karna.

1. Thrombocytopenic Purpura Ka Treatment

  • Immune Thrombocytopenic Purpura (ITP):

    • Corticosteroids: Immune system ko suppress karke platelet count ko badhate hain.

    • Intravenous Immunoglobulin (IVIg): Severe cases mein platelet destruction ko rokne ke liye use hota hai.

    • Splenectomy: Chronic ITP mein, spleen ko hatana padta hai agar treatment se results nahi milte.

  • Thrombotic Thrombocytopenic Purpura (TTP):

    • Plasmapheresis (plasma exchange): Ye first-line treatment hai, jo ADAMTS13 enzyme ko replace karta hai aur harmful antibodies ko remove karta hai.

    • Steroids aur immunosuppressive agents (cyclophosphamide) bhi severe cases mein use kiye jaate hain.

  • Heparin-Induced Thrombocytopenia (HIT):

    • Heparin ko band karna aur alternative anticoagulants (argatroban) dena.

2. Non-Thrombocytopenic Purpura Ka Treatment

  • Vasculitis:

    • Corticosteroids aur immunosuppressive drugs (cyclophosphamide, methotrexate) ka use severe vasculitis mein hota hai.

  • Scurvy:

    • Vitamin C supplementation.

  • Drug-Induced Purpura:

    • Causative drug ko discontinue karna aur supportive treatment.

3. Supportive Management

  • Blood Transfusions: Agar bleeding ya anemia severe ho, toh transfusion ki zaroorat ho sakti hai.

  • Pain management aur wound care.

  • Monitoring organ function, especially TTP aur vasculitis mein, taaki complications ko early detect kiya ja sake.