Correct!
5. All of the above

LIP may be associated with autoimmune and lymphoproliferative disorders, including rheumatoid arthritis, Hashimoto thyroiditis, myasthenia gravis, pernicious anemia, autoerythrocyte sensitization syndrome, chronic active hepatitis, common variable immunodeficiency, Sjögren syndrome, allogeneic bone marrow transplantation, lupus, and lymphoma. LIP has also associated with infection via human immunodeficiency virus (HIV), Epstein-Barr virus, and human T-cell leukemia virus (HTLV).

The major principle is to treat the underlying disease. LIP patients who are asymptomatic and physiologically unaffected patients may not require treatment. Symptomatic patients may require supportive care and immunosuppressives, chiefly corticosteroids. Occasionally, cytotoxic therapy has been used.

Our patient was found to have hypogammaglobulinemia and started on weekly intravenous immunoglobulin (IVIG).

She had frequent infections including a diagnosis of Mycobacterium avium-intracellulare over the next 2 years despite the immunoglobulin therapy. She underwent splenectomy for ITP. Biopsies of the spleen, omentum and liver revealed reactive follicular lymphoid hyperplasia with non-necrotizing granulomas.

Which of the following diseases associated with LIP is likely in our patient? (Click on the correct answer to proceed to the fifth and final panel)

  1. ADA deficiency
  2. Common variable immunodeficiency
  3. Severe combined immunodeficiency (SCID)
  4. Wiskott-Aldrich syndrome (WAS)
  5. X-linked agammaglobulinemia (Bruton disease; XLA)

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