D69.41

Billable

Evans syndrome

HCC Category Mapping

V28HCC 112 — Von Willebrand Disease and Other Coagulation Defects

0.247

V24HCC 48 — Coagulation Defects and Other Specified Hematological Disorders

0.209

ESRDHCC 48 — Coagulation Defects and Other Specified Hematological Disorders

0.000

What This Code Means

A rare autoimmune condition where the body simultaneously destroys both red blood cells and platelets, causing anemia and bleeding problems.

Coding Tips

•Ensure both hemolytic anemia and thrombocytopenia are documented and coded separately
•Distinguish from ITP alone by confirming evidence of hemolysis

Clinical Significance

Evans syndrome is an autoimmune condition characterized by the simultaneous or sequential combination of immune thrombocytopenic purpura and autoimmune hemolytic anemia, sometimes with autoimmune neutropenia. It is more resistant to treatment than isolated ITP or autoimmune hemolytic anemia and carries a higher morbidity and mortality. The condition may be primary or secondary to lymphoproliferative disorders or autoimmune diseases.

Documentation Requirements

✓Document concurrent thrombocytopenia with positive antiplatelet antibodies AND autoimmune hemolytic anemia with positive direct antiglobulin test (Coombs test).
✓Record reticulocyte count, LDH, haptoglobin, and bilirubin confirming hemolysis alongside platelet counts.
✓Note treatment history including response to corticosteroids, IVIG, rituximab, and splenectomy.

Commonly Confused Codes

D69.3 (Immune thrombocytopenic purpura) — isolated ITP without hemolytic anemia component D59.1 (Other autoimmune hemolytic anemias) — isolated AIHA without thrombocytopenia D65 (DIC) — may cause both anemia and thrombocytopenia but through consumptive mechanism.

Code Hierarchy

└D69Purpura and other hemorrhagic conditions └D69.4Other primary thrombocytopenia └D69.41Evans syndrome

└D69.41Evans syndrome