Coagulopathies¶
Jason Jones
Overview¶
- Step one: determine if platelet vs coagulation disorder
- Platelet Disorders: Skin/mucous membrane bleeding; Petechiae; Often mild bleeding following surgery and tends to immediately follow surgery
- Coagulation defect: Bleeding deep in soft tissues (muscles/joints); hemarthroses, hematomas; tendency to have delayed bleeding after surgery that can be severe
- Coagulopathies can be divided into Hereditary vs Acquired causes
Hereditary¶
Hemophilia A (Factor VIII Deficiency) and Hemophilia B (Factor IX Deficiency)¶
- Inherited in X-linked Recessive pattern
- Diagnosis: Isolated prolonged PTT with normalization upon mixing study
- Management: purified/recombinant Factor VIII or IX. Desmopressin for mild disease
- Consult benign hematology every time these patients are admitted
von Willebrand Disease (vWD)¶
- Can be hereditary (common) or acquired
- Abnormal quantity or function of von Willebrand Factor (vWF) needed for platelet function
- Type 1 (most common): Quantitative defect. Low quantity, normal function of vWF
- Type 2: Qualitative defect. Normal quantity, abnormal function of vWF.
- Type 3 (rare): Complete absence of vWF, phenotypically similar to hemophilia A
- Order in Epic: vW Profile = vWF Ag, Factor VIII Activity, Ristocetin Cofactor Activity
- Management: Desmopressin (DDAVP) can be useful as prophylaxis or treatment; most patients are treated with factor concentrate replacements
Acquired¶
Coagulation Factor Inhibitors¶
- Associated with autoimmune disease (paraneoplastic vs. autoantibody)
- Seen in hemophilia due to frequent treatments with recombinant factors (alloantibody)
- Diagnosis: Elevated PTT that does not normalize with mixing study
- Management: Immunosuppression with steroids; cyclophosphamide ± rituximab
- Consult Hematology always (rare disorder with major bleeding complications)
Vitamin K Deficiency¶
- Caused by malnutrition, liver disease, or iatrogenic with warfarin
- Diagnosis: elevated PT, if severe may have prolonged PTT as well
- Management: Replace vitamin K
Last update:
2022-06-25 02:05:01