Primary splenic involvement in kaposiform lymphangiomatosis: A case report

Ethan Covil ¹, Robert Gates ²

⁰University of South Carolina School of Medicine Greenville, 607 Grove Rd, Greenville, SC 29605, United States of America.

International Journal of Surgery Case Reports +

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January 22, 2025

View abstract on PubMed

Summary

Kaposiform hemangiomatosis (KLA) can cause severe coagulopathy. This case highlights KLA isolated to the spleen as a cause of severe consumptive coagulopathy in an infant.

Area Of Science

Pediatric Hematology
Vascular Anomalies
Pediatric Surgery

Background

Kaposiform hemangiomatosis (KLA) is a rare lymphatic anomaly with high mortality.
Previously, KLA was primarily associated with thoracic involvement.
Splenic involvement in KLA is less common and often secondary.

Observation

An 18-month-old female presented with sudden petechiae, bruising, anemia, and thrombocytopenia.
The patient rapidly progressed to disseminated intravascular coagulopathy.
Splenomegaly was noted, prompting surgical consultation.

Findings

Emergent splenectomy stabilized the patient's condition.
Pathologic evaluation of the spleen confirmed KLA.
No thoracic or mediastinal KLA involvement was detected.

Implications

This is the first reported case of KLA isolated to the spleen causing severe consumptive coagulopathy.
KLA should be considered in the differential diagnosis of sudden, severe thrombocytopenia and coagulopathy in children.
Early recognition and management of KLA are crucial for improving patient outcomes.

Keywords:

Consumptive coagulopathy Kaposiform Lymphangiomatosis Splenomegaly