Case Study

A 60-year-old woman complained of gastric pain and vomiting for 2 weeks. She had no fever, but a CAT scan was ordered and showed a slightly enlarged spleen. An enlarged lymph node was also discovered. The patient complained of severe itching, redness and scaling of the skin, and pitting edema. A bone marrow showed a hypocellular architecture with increased fat.

Laboratory findings are as follows: WBC 39.0 X 109/L Differential: RBC 4.25 X 1012/L Segs 29%

Hgb 11.7 g/dL Lymphs 67%

Hct 38% Eosinophils 4%

MCV 89 fL Platelets normal

MCH 27.5 pg Technologist note: lympho-

MCHC 30.6% cytes appear abnormal with rounded, clefted, folded or bilobed nucleus; vacuoles in some cells

Considering the patient's symptoms, which are unusual, the increased white count and the differential reversal, what are the diagnostic possibilities?

Insights to the Case Study

Relative lymphocytosis is usually reported in conditions like infectious mononucleosis, hepatitis virus infection, or cytomegalovirus infection. These lymphocytes, however, showed a distinct morphology with a large cell and a small cell variant. Nuclear clefting or folding may be seen in lymphoma cells, but lymphoma cells rarely have vacuoles. An additional finding is that these cells were very large, some up to 20 pm, and the clefting manifestation is very pronounced. When clinical characteristics are included, the most likely diagnosis in this case is Sezary syndrome, a rare type of T-cell lymphoma. This disorder usually has serious skin manifestations, as shown in our patient, combined with an elevated white count and rising lymphocyte count. The abnormal lymphocyte morphology usually causes confusion when performing a differential due to the unusual nuclear manifestations of these cells. Sezary cells are usually confirmed by immunophenotyping and are usually CD4-positive T lymphocytes. The life expectancy for a patient with this condition is around 5 years.

(Adapted from Hematology Problem, November 1981, American Journal of Medical Technology.)

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