A 30-Year-Old Man with Fever and Lymphadenopathy.

A 30-year-old man was seen in an outpatient clinic at this hospital because of fever and lymphadenopathy.

The patient had been well until approximately 2 weeks before presentation, when an enlarging, tender lump developed at the posterior base of the neck on the right side. Two days before presentation, fever to a temperature of 39.4°C, a mild headache, myalgias, chills, and fatigue developed. He took ibuprofen, but his condition did not improve, and he came to this hospital for evaluation.

The patient reported no history of sore throat, coryza, or earache. He had had a low hemoglobin level in the past but was otherwise healthy. He reportedly had had a negative tuberculin skin test in the past, and he had not received an influenza vaccine during the previous year. He took no other medications and had no known allergies.

He was born in India and came to the United States 4 years previously to attend school; his most recent visit to India was 6 months before presentation. He worked in an office and lived with a roommate. He was not sexually active and had no known exposures to sick contacts, animals, or blood products. He had stopped smoking 2 years before this presentation, drank alcohol occasionally, and did not use illicit drugs. His parents had diabetes mellitus; there was no family history of autoimmune or connective-tissue diseases.

On examination, the temperature was 38.9°C, the blood pressure 129/80 mm Hg, and the pulse 104 beats per minute. A group of five tender lymph nodes, each approximately 1 cm in diameter, was present in the posteroinferior cervical chain on the right side; the lymph nodes in the posterior cervical chain on the left side and in both inguinal regions were nontender, and there were no abnormal lymph nodes in the supraclavicular or axillary regions.

A systolic ejection murmur (grade 1 out of 6) was heard at the cardiac base; the remainder of the examination was normal. During evaluation, the temperature rose to 39.5°C and was associated with chills. Blood levels of glucose, total protein, albumin, and globulin were normal, as were results of tests of liver and renal function; testing for liver and renal function and rapid tests for streptococcal pharyngitis and influenza virus were negative. A blood culture was sterile. The administration of acetaminophen alternating with ibuprofen was recommended, as were fluids and rest. The patient returned home but was advised to return to the outpatient clinic if his condition did not improve.

Cytologic smears of the fine-needle aspirate showed a mixed population of lymphocytes and histiocytes, including phagocytic histiocytes with crescentic nuclei. Two days later, the patient returned to the clinic. He reported decreased appetite, chills, sweats related to fevers, mild headaches and body aches, and a new nonproductive cough.

On examination, the lymph nodes on the right side were slightly larger than on the previous examination and were slightly confluent; the lymph nodes on the left side and a submental lymph node were soft and mobile. Bronchial breath sounds were heard over the right side of the chest anteriorly, and the remainder of the examination was unchanged. Hemoglobin electrophoresis and DNA sequence analysis revealed hemoglobin D Punjab, an abnormal variant of the β-globin chain of hemoglobin, at a level of 91.8%, as well as a hemoglobin A level of 0% (reference range, 95.8 to 98.0), a hemoglobin A₂ level of 7.0% (reference range, 2.0 to 3.3), and a hemoglobin F level of 1.2% (reference range, 0.0 to 0.9); The patient declined testing for the human immunodeficiency virus (HIV).

Dr. Daniel F. Boyer: A fine-needle aspiration biopsy of an enlarged cervical lymph node was performed, and the aspirate showed a mixed population of lymphocytes, histiocytes, and occasional plasma cells. Some of the histiocytes contained cellular debris and angular, crescentic nuclei. The polymorphous composition of the aspirate and the mature appearance of the lymphocytes were suggestive of a reactive process. Gram’s and acid-fast staining of the aspirate revealed no organisms. Flow cytometry of the aspirate showed no immunophenotypic abnormalities.

Dr. Rosenbluth: A blood culture and a culture of the aspirate were sterile. A chest radiograph was normal. The patient returned home again, with instructions to return to the clinic if fevers persisted. A coronal reformatted image from a contrast-enhanced CT scan of the neck shows multiple bilateral enhancing cervical lymph nodes, the largest measuring 9 mm in diameter (Panel A, arrow). A coronal reformatted image from a CT scan of the chest (Panel B) confirms the presence of scattered small axillary lymph nodes bilaterally (white arrowheads) and subcarinal lymph nodes, as well as splenomegaly.

Two days later, the patient reported persistent fevers and a dull headache and was admitted to the hospital. He reported no night sweats, weight loss, rhinorrhea, neck rigidity, neck pain, vision changes, weakness, or numbness and no respiratory, gastrointestinal, or polymorphous composition.

On examination, the blood pressure was 106/58 mm Hg, and the pulse 104 beats per minute; the respirations were 20 breaths per minute, with an oxygen saturation of 98% while the patient was breathing ambient air.

There was a superficial ulcer on the right side of the lower lip, and the spleen tip was palpable approximately 3 cm below the costal margin; the remainder of the examination was unchanged. Blood levels of lactic acid, C3, C4, haptoglobin, creatine kinase, and thyrotropin were normal; testing for rheumatoid factor and antinuclear antibodies was negative. Urinalysis revealed yellow, clear fluid, with 1+ occult blood, trace albumin, and few squamous cells per high-power field, and was otherwise normal. Fluids were administered intravenously, and another blood sample was obtained for culture.

The maximal daily temperature was 40.7°C on the first day. On the second day, the temperature rose to 40.8°C. Dr. Victorine V. Muse: Computed tomographic (CT) scans of the neck and chest obtained after the administration of contrast material showed lymph nodes measuring up to 9 mm in diameter in the submental, submandibular, and jugular chains and at the posterior cervical level; mediastinal and hilar lymph nodes, the largest, at the subcarinal level, measuring 9 mm in diameter; bilateral, clustered axillary lymph nodes measuring up to 10 mm in diameter; a small, anterior pericardial effusion; a soft-tissue nodule, 4 mm in diameter, in the left lower lobe; mild centrilobular emphysema, predominantly in the upper lobe; and occasional subcentimeter cystic lucencies, predominantly in the paramediastinal lingula and right upper lobe.

CT scans of the abdomen and pelvis obtained after the administration of contrast material showed splenomegaly, measuring 16.5 cm in the maximal craniocaudal dimension (normal range, <14 cm); a cystic structure, 3.5 cm by 2.6 cm, in the interpolar region of the right kidney; and no evidence of lymphadenopathy in the abdomen or pelvis.

PHL.