kaposiform lymphangiomatosis

ORPHA: 464329

Overview

generalized lymphatic anomaly characterized by kaposiform spindled lymphatic endothelial cells

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with kaposiform lymphangiomatosis, sourced from HPO and Orphanet clinical annotations.

Abnormality of the respiratory systemDyspneaOsteolysisAbnormal skeletal morphologyHypofibrinogenemiaCoughAbnormal spleen morphologyEpistaxisAbnormality of the neckAbnormal scapula morphologyBruising susceptibilityHepatosplenomegalyPancreatic cystsSplenomegalyThrombocytopeniaFeverAbnormal femur morphologyRecurrent long bone fracturesAbnormal ischium morphologyAbnormal form of the vertebral bodiesAbnormality of the cervical spineExercise intoleranceAbnormal sacrum morphologyMultiple renal cystsSubconjunctival hemorrhageAbnormal humerus morphologyEcchymosisAbnormal pelvis bone morphologyEpidural hemorrhageMetrorrhagiaAbnormal thoracic spine morphologyChest painLymphangiomaEnlarged kidneyAnemiaHemoptysisAbnormal skull base morphologyPapillomaAbnormal lung morphologyPleural effusionAbnormal mediastinum morphologyAbnormality of the lymphatic systemAbnormal lymphatic vessel morphologyAbnormal thorax morphologyPericardial effusionAbnormal bleeding

Classification & Codes

Orphanet Code

ORPHA:464329
kaposiform lymphangiomatosis
OrphanetORPHA:464329
Treatments0 drug(s)
Symptoms on record46 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO