D69.42

Billable

Congenital and hereditary thrombocytopenia purpura

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

An inherited bleeding disorder present from birth where platelet counts are abnormally low due to genetic factors.

Coding Tips

•Document family history and genetic testing results if available
•Specify the type of hereditary thrombocytopenia if identified (e.g., May-Hegglin anomaly, Wiskott-Aldrich syndrome)

Clinical Significance

Congenital and hereditary thrombocytopenia purpura encompasses inherited conditions with reduced platelet production or survival, including Wiskott-Aldrich syndrome, May-Hegglin anomaly, Bernard-Soulier syndrome (when thrombocytopenic), thrombocytopenia-absent radius (TAR) syndrome, and MYH9-related disorders. These conditions vary widely in severity and associated features.

Documentation Requirements

✓Document the specific inherited thrombocytopenia when identified, platelet count and mean platelet volume (some conditions have characteristically large or small platelets), family history of thrombocytopenia, genetic testing results, and associated syndromic features.
✓Record any associated conditions such as immunodeficiency (Wiskott-Aldrich) or skeletal abnormalities (TAR).

Code First

congential or hereditary disorder, such as:
thrombocytopenia with absent radius (TAR syndrome) (Q87.2)

Commonly Confused Codes

D69.3 (Immune thrombocytopenic purpura) — acquired autoimmune condition frequently misdiagnosed in patients with unrecognized inherited thrombocytopenia D69.49 (Other primary thrombocytopenia) — may overlap clinically D69.6 (Thrombocytopenia, unspecified) — should not be used when an inherited cause is documented.

Code Hierarchy

└D69Purpura and other hemorrhagic conditions └D69.4Other primary thrombocytopenia └D69.42Congenital and hereditary thrombocytopenia purpura

└D69.42Congenital and hereditary thrombocytopenia purpura