Idiopathic CD4+ lymphocytopenia (ICL) is a rare disorder that can predispose otherwise immunocompetent individuals to life-threatening opportunistic infections. We present a case of a human immunodeficiency virus (HIV)-negative patient with ICL who presented with recurrent cryptococcosis.

Case Presentation

A 39-year-old Caucasian, HIV-negative male with a past medical history of stroke 13 years prior to admission and an episode of cryptococcal meningitis 18 months prior to admission presented to the hospital with generalized weakness for 7 days. The patient had a 10 pack-year smoking history and no significant environmental or occupational exposures. On admission, vital signs were significant for a temperature of 101.1oF, a heart rate of 117 beats per minute, and a blood pressure 93/75 mm Hg. Physical exam was remarkable for an ill-appearing male with dry mucous membranes and tachycardia.

Initial laboratory studies were significant for a white blood count of 3.14 cells/μL (normal range = 4.5-11 cells/μL), hemoglobin of 8.7 g/dL (normal range = 13.5-17.5 g/ dL), and creatinine of 5.2 mg/dL (normal range = 0.6-1.2 mg/dL). Blood cultures were positive for Cryptococcus neoformans. Subsequent laboratory studies revealed a serum cryptococcal antigen titer of 1:256 and a CD4+ count of 227 cells/μL (normal range = 410-1590 cells/ μL). The CD4+ count was 98 cells/μL when the patient was diagnosed with cryptococcal meningitis 18 months prior. Enzyme-linked immunosorbent assays for HIV were negative. Anti-nuclear antibody titer was 1:80 and anti-double stranded DNA, anti-Smith, anti-SSA, anti-SSB , and anti-ribonucleoprotein antibodies were negative. Lumbar puncture (LP) showed a normal opening pressure and was negative for cryptococcal infection.

Chest X-ray revealed a right pleural mass. Subsequent CT of the thorax revealed a 6.3 x 3.3 cm pleural-based mass in the right upper lobe of the lung (Figure 1). Due to suspicion for a pulmonary malignancy, a Technetium bone scan was performed and found increased uptake in the right femur and left humerus. X-ray of the right femur showed well-circumscribed lytic lesions in the mid-femoral diaphysis (Figure 2). CT-guided biopsy of the pleural mass and the femur lesion revealed narrow-based, budding yeast that stained Gomori methenamine silver (Figure 3a) and a capsule that stained red with mucicarmine (Figure 3b), consistent with a diagnosis of cryptococcosis.