Hyphen

You meet this patient with a rare autosomal recessive disease. She has a history of recurrent severe bacterial infections including pyoderma, skin abscesses, and ulcerations. They are often caused by Staphylococcus, Group A Streptococcus, and Pneumococcus. Her dentist follows her for gingivitis, oral ulcerations, and periodontal disease. She has had a few episodes of respiratory tract infections as well. On review of systems, the patient reports bruising easily and abnormal musocal bleeding.

On exam, you note hepatosplenomegaly and lymphadenopathy. Unfortunately, as she gets older, her exam may show weakness, sensory deficits, peripheral neuropathy, ataxia, tremors, cranial nerve palsies, and seizures. Laboratory tests show neutropenia. Blood smear is shown below.

Challenge: What is this hyphenated diagnosis?

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