Normal hemostasis has three steps: vascular spasm, a platelet plug (platelets adhere to injured vessel wall with the help of von Willebrand factor), and the coagulation cascade, which forms a stable fibrin clot. Fibrinolysis later dissolves the clot. A bleeding disorder results from too few or poorly functioning platelets, deficient or inhibited clotting factors, or excessive consumption of both.

Key conditions

• ITP — autoimmune destruction of platelets; isolated low platelet count
• TTP — severe deficiency of the enzyme ADAMTS13 → platelet microthrombi in small vessels → thrombocytopenia, hemolytic anemia, and organ ischemia (brain, kidney). Rare but fatal without prompt treatment
• Hemophilia — X-linked recessive; affects mainly males; severity depends on factor level
• von Willebrand disease — the most common inherited bleeding disorder; affects both sexes. vWF helps platelets stick and carries and protects factor VIII
• Vitamin K deficiency — the liver needs vitamin K to make factors II, VII, IX, and X. Causes: poor intake, malabsorption, biliary obstruction, prolonged antibiotics, newborns without prophylaxis, warfarin
• Liver disease (cirrhosis) — reduced production of most clotting factors and low platelets (splenic sequestration), plus portal hypertension → esophageal varices
• DIC — a triggering illness (sepsis, trauma, obstetric emergencies, acute promyelocytic leukemia, cancer, burns, snakebite envenoming) causes widespread clotting in small vessels. Platelets and clotting factors are consumed, and fibrinolysis is activated → simultaneous thrombosis and bleeding

• Skin: petechiae, purpura, ecchymoses (bruises), new bruises during anticoagulation
• Mucosa: gum bleeding, nosebleeds, conjunctival hemorrhage
• Joints and muscles: warm, swollen, painful joint with limited movement (hemarthrosis — knees, elbows, ankles); tingling or pain in a joint may be the first sign
• Hidden bleeding: hematuria, melena or hematemesis, heavy menses, headache or change in consciousness (intracranial hemorrhage), back or flank pain (retroperitoneal)
• Circulatory signs of blood loss: tachycardia, hypotension, decreasing urine output, pallor, restlessness
• DIC: oozing from IV sites, incisions, and mucous membranes; plus signs of clotting — cyanotic fingers and toes, oliguria, confusion, dyspnea
• TTP: confusion or stroke-like signs, fever, jaundice, dark urine, kidney injury with low platelets

Summary patterns

Hemophilia

• Replace the missing factor: factor VIII concentrate for hemophilia A, factor IX for hemophilia B — for acute bleeds (give first, as early as possible) and before surgery or dental procedures to raise the factor level (near normal for major surgery) and prevent excessive bleeding
• Prophylaxis with regular factor infusions or emicizumab (subcutaneous, hemophilia A). Emicizumab alters aPTT-based lab results and has a thrombotic microangiopathy risk when combined with activated prothrombin complex concentrate
• Desmopressin (DDAVP) raises factor VIII and vWF in mild hemophilia A and type 1 vWD — risks: hyponatremia and seizures (limit fluids for 24 hours), flushing, tachyphylaxis with repeated doses. Not effective in severe hemophilia A or in hemophilia B; avoid in type 2B vWD (worsens thrombocytopenia)
• Tranexamic acid (antifibrinolytic) for mouth, nose, and menstrual bleeding; avoid in active thrombosis and with hematuria from the upper urinary tract
• Gene therapy is available for some adults in some countries

von Willebrand disease: desmopressin (type 1), vWF-containing concentrates, tranexamic acid, hormonal therapy for heavy menses.

ITP: treat when platelets are below about 30,000/µL or with bleeding. Corticosteroids first-line (hyperglycemia, hypertension, mood change, infection); IV immunoglobulin for rapid rise (headache, aseptic meningitis, kidney injury, thrombosis); thrombopoietin receptor agonists (eltrombopag — liver toxicity; romiplostim); rituximab; splenectomy (vaccinate beforehand).

TTP: medical emergency — plasma exchange, corticosteroids, and caplacizumab. Platelet transfusion is generally avoided unless bleeding is life-threatening.

HIT: stop all heparin (including flushes and heparin-coated catheters), start a non-heparin anticoagulant (e.g., argatroban). HIT causes clotting, not mainly bleeding. Do not start warfarin until platelets recover (risk of venous limb gangrene); avoid platelet transfusion unless bleeding.

Vitamin K deficiency: vitamin K (phytonadione) by mouth or slow IV infusion (IV can cause rare anaphylactoid reactions); subcutaneous absorption is unreliable and IM injection risks hematoma in adults. PT/INR improves within about 12–24 hours. For life-threatening bleeding, add prothrombin complex concentrate (PCC) or plasma.

Anticoagulant reversal

DIC: treat the underlying cause (antibiotics and source control for sepsis, delivery for obstetric causes). Support with blood products when bleeding or before procedures: platelets (if below about 50,000/µL with bleeding), fresh frozen plasma for prolonged PT/aPTT, and cryoprecipitate for low fibrinogen (commonly below 150 mg/dL (1.5 g/L)). Heparin may be used when thrombosis predominates.

Blood products

• Fresh frozen plasma (FFP) — contains all clotting factors; used for multiple factor deficiencies (DIC, liver disease, massive transfusion, warfarin reversal when PCC is unavailable). ABO-compatible; watch for volume overload
• Cryoprecipitate — fibrinogen, factor VIII, vWF, factor XIII
• Platelets — stored at room temperature; infuse promptly; do not refrigerate
• PCC — concentrated factors II, VII, IX, X; faster and smaller volume than plasma for warfarin reversal

Liver disease and variceal bleeding: acute bleeding from esophageal varices is managed with airway protection, careful volume replacement with restrictive transfusion (Hb about 7 g/dL (70 g/L)) because overtransfusion raises portal pressure, a vasoactive drug (octreotide), antibiotic prophylaxis, and endoscopic variceal band ligation as the first-line endoscopic treatment.

Listed in priority order.

1. Active bleeding — apply direct pressure; assess airway (oral or upper GI bleeding), vital signs, and level of consciousness; large-bore IV access; prepare blood products and factor concentrates; notify the provider
2. Hemophilia joint bleed — give factor replacement first, then rest, immobilize, apply cold, elevate; after bleeding stops, gradual range-of-motion exercises to prevent contractures
3. Recognize bleeding during anticoagulation — new bruising, petechiae, blood in urine or stool, headache, falling Hb or BP; hold the infusion per protocol and report. Monitor aPTT (heparin) and PT/INR (warfarin)
4. DIC — monitor all puncture sites, urine, stool, and wounds; watch for organ ischemia (cold, dusky digits; oliguria; confusion); minimize punctures; gentle oral care
5. Bleeding precautions — soft toothbrush, electric razor, no IM injections, no rectal temperatures or enemas, apply pressure 5–10 minutes after venipuncture, pad side rails, prevent falls, stool softeners to avoid straining
6. Transfusion safety — verify product and client identity with two people; observe closely during the first 15 minutes; stop the transfusion for any reaction
7. Desmopressin — monitor sodium, restrict fluids as ordered, observe for headache or confusion

• Avoid aspirin and NSAIDs (including ibuprofen, naproxen) when platelets are low or with hemophilia or vWD — use acetaminophen for pain
• Report nosebleeds lasting more than 10 minutes, blood in urine or stool, black stools, severe headache, joint swelling, or heavy menses
• Use a soft toothbrush and electric razor; maintain dental health; tell dentists and surgeons about the disorder before any procedure
• Wear medical identification
• Hemophilia: learn home factor infusion; treat bleeds early; choose low-impact activities (swimming, walking, cycling); avoid contact sports; vaccines should be given subcutaneously when possible
• Warfarin: regular INR checks, consistent vitamin K intake, consult before any new drug or herbal product
• Genetic counseling for hemophilia (X-linked: carrier mothers, affected sons)
• ITP: report new petechiae or bleeding; avoid activities with high injury risk while platelets are low

• Platelet disorders → petechiae and mucosal bleeding; factor disorders → hemarthrosis and deep bleeding
• Hemophilia A = factor VIII, B = factor IX; aPTT prolonged, PT and platelets normal; X-linked, males
• Joint bleed in hemophilia: factor concentrate first, then rest, ice, elevation
• Preoperative factor replacement prevents surgical bleeding
• vWD = most common inherited bleeding disorder; aPTT may be prolonged; desmopressin for type 1
• Warfarin → PT/INR; reversal with vitamin K ± PCC. Heparin → aPTT; reversal with protamine
• Vitamin K deficiency → prolonged PT → vitamin K replacement
• FFP replaces multiple clotting factors; cryoprecipitate replaces fibrinogen
• DIC = clotting and bleeding at the same time; low platelets and fibrinogen, high D-dimer; treat the cause
• New bruising on a heparin infusion = bleeding sign — report
• Platelets very low → no aspirin, bleeding precautions
• Variceal bleeding → endoscopic band ligation