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Clinical Vascular Disorders




Senile Pupura Purpura Simplex Scurvy Hereditary Hemorrhagic Telangiectasia

Age-related decrease in tensile strength of small vessels Estrogen/Progesterone-related Vitamin C Abn hydroxyproline, collagen AD Absent elastin fibers in venules Endoglin Activin-R-Like kinase Acquired (Viral, Drugs) IgA Vasculitis Leukocytoclastic vasculitis Fibrinoid necrosis of vessel

Elderly Ecchymoses Hematoma from IV, draws Menstruating women Perifollicular petechiae Epistaxis (1° presentation) Telangiectasias: lips, tongue, Telangiectasias of skin and conjunctivae mucous membranes, rectal A/V Malformations: nasal bleed, dyspnea, intracranial mucosa, skin, GI, lung, brain bleed Petechiae, Ecchymoses Palpable purpura GI Bleed Renal failure Quantitative Platelet Disorders Petechiae, ecchymoses, epistaxis, gingival, GI, GU, BM: MegaK intracranial Acute, < 6mos, children Chronic, > 6mos, adults Fever (#3) Petechiae Ecchymoses Purpura Neurologic (CNS thrombi) (#4) Renal failure (#5) Acute bloody diarrhea No neurologic symptoms

Henoch-Shonlein Pupura




Platelet Autoantibodies to Ib V IX or IIb IIIa Fc region of MØ Cleared by spleen 2° to viral, SLE, lymphoma 10% genetic, 90% autoantibody ADAMTS-13 ULvWF (Weibel-Palade bodies on endothelial cells) Microvascular Plt Agglutination 2° to SLE, Plavix, HIV, pregnancy, HSCT Plt Aggregation in kidney 2° to ECOL O157:H7 Shiga-like toxin

Plt BT Anti-Plt IgG

Glucocorticoids Splenectomy Rituximab

Hyaline microthrombi in kidney (+Plt ØFibrin) Schistocytes (#1)

Plt (< 20) (#2) LDH (10x N) Creat

Urgent plasma exchange FFP (ADAMTS-13) Rituximab (for autoimmune cases)

Hyaline microthrombi in kidney (+Plt +Fibrin) Schistocytes

Plt Creatinine

Supportive care

Qualitative Platelet Disorders Bernard-Soulier Glanzmann Thrombasthenia Ar Ib V IX deficiency Ar IIb IIIa deficiency Plt Ø aggregation with Ristocetin Normal Plt

+ aggregation with

Disease Pathogenesis Clinical (Qualitative Platelet Disorders) Pathologic Labs Treatment Storage Pool Disease (Gray Platelets) Storage Pool Disease Inherited granule deficiency Inherited Dense granule deficiency ASA: Irreversible COX-1 inhibitor NSAIDS: Reversible COX inhibitors Plt Function Inhibitors: PI2. viruses and subsequent cirrhosis and/or malignancy Hemarthroses Hematomas Eccymoses Uniform severity between generations Degenerative arthritis. PT. ADP Ø Response: collagen Confirmation by EM Drugs Kidney Failure Uremic Bleeding Coagulation Disorders Hemodialysis Hemophilia A Xr 1:10. VIIIi.000 Males IX deficiency Hemarthroses Hematomas Eccymoses Uniform severity between generations Degenerative arthritis.000.000 (MOST COMMON INHERITED COAGULATION DISORDER) Males VIII deficiency Hemophilia B Xr 1:60. 2-5%. Phenolic Acids Mild Moderate bleeding Mild Moderate bleeding No granules seen on perip smear Confirmation by EM Aggregation studies: Ø 2° wave: epi. VIIIi. fatal bleeds.000 XIII deficiency Defective fibrin cross-linking (No covalent D=D) Normal BT. NO. fatal bleeds. >5%) Recombinant VIII Cryo Recombinant VIIa (for VIIIi) PTT Normal BT Corrected PTT 1:1 Mix Normal PT IX Activity XIII Deficiency Ar Rare .1:1. viruses and subsequent cirrhosis and/or malignancy Bleeding Delayed wound healing Persistent umbilical stump bleed PTT Normal BT Corrected PTT 1:1 Mix Normal PT VIII Activity ( 1%. PTT Urea Solubility .

PTT Low -2-AP activity . malabsorption Hemorrhagic Disease of the Newborn II.2. malnutrition. RA. PE Bleeding: rectal.000 (MOST COMMON INHERITED BLEEDING DISORDER) Variable age of onset Qualitative or Quantitative Type 1: Partial Quant (AD) Type 2: Qualitative Type 3: Complete Quant (Ar) Idiopathic (>60yo) 2° to SLE. extremity infarct Venous thrombosis: legs.PT. etc Fibrinolytic Disorders Bleeding following trauma or surgery Spontaneous bleeding Bleeding following trauma or surgery FFP PAI-1 Deficiency -2-Antiplasmin Deficiency Ar Rare AD Rare Homozygous more severe Normal BT. VII.2) VIIIi Recombinant VIIa Prednisone Cyclophosphamide DIC Arterial thrombosis: stroke.Disease Pathogenesis Clinical (Coagulation Disorders) Pathologic Labs Treatment vWD 1-5 per 1. retained dead fetus 2° to antibiotics. M3. hematuria. PTT Low PAI-1 activity Normal BT. postpartum. VP sites Find and treat cause Supportive therapy with replaced blood products Vit K Mild Severe bleeding GI. bleeding after tooth extraction Type 3: Hemarthroses and hematomas Variable severity between generations Mild Severe bleeding Spontaneous resolution or chronic BT (Use PFA) PTT (50%) vWF activity (1. Intracranial. GU. IX. VP. carcinoma.3) Normal vWF Ag (2) PTT Normal BT & PT NON-corrected PTT 1:1 Mix VIII Activity Plt D-dimer Hyaline microthrombi in microvessels (+Plt +Fibrin) PT and/or PTT Fbg Schistocytes PT (FIRST INDICATOR OF VitK) PTT (Severe) Normal BT PT PTT (Severe) Normal BT Cryo (1. trauma. GU. placental abruption. hemophilia A Release of Tissue Factor Overactivation of coagulation system and 2° fibrinolysis Consumption of coagulation factors and platelets 2° to bacterial sepsis. PT. PS. Intracranial.3) vWF Ag (1. PC 2° to any disease causing hepatocellular damage All coagulation factors are made by hepatocytes Mucocutaneous petechiae and ecchymoses.3) Desmopressin (1. etc Vit K (SQ) 2-6hr FFP more rapid Liver Disease Mild Severe bleeding GI. petechiae. VP.2. X.