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

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

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

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)

Last update: 2022-06-25 02:05:01