Autoimmune lymphoproliferative syndrome physical examination

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Editor-In-Chief: David Teachey, MD [1]

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Overview

Common physical examination findings of Autoimmune lymphoproliferative syndrome(ALPS) include lymphadenopathy, Hepatomegaly, or splenomegaly. The majority of patients(80%) have enlarged, palpable, non -tender lymph nodes for an extended period of time. Cervical, axillary, inguinal lymphadenopathy are mostly found. But preauricular, submental, epitrochlear, mediastinal, and retroperitoneal nodes are detected occasionally. Moderate to massive splenomegaly is evident in 85% of patients with ALPS. Minor hepatomegaly is also a common finding. Lymphadenopathy, splenomegaly, hepatomegaly improve with age.

Physical Examination

Skin

• Pallor
• Rash
• Petechiae

Head

• Lymphadenopathy: >90% of patients present with chronic non-malignant lymphadenopathy. It can be mild to severe, affecting multiple nodal groups. Most commonly presents with massive non-painful hard cervical lymphadenopathy

Abdomen

• Splenomegaly: >80% of patients present with clinically identifiable splenomegaly. It can be massive.
• Hepatomegaly: 30-40% of patients have enlarged livers.