Page 19 - Delaware Medical Journal - November 2015
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CASE REVIEW
IntroductionIn 1985, a hematological abnormality present in the marrow, spleen, and liver characterized by an abundance of large lymphocytes with azurophilic granules was named by Loughran et al.2
percent of all chronic lymphoproliferative disease in North America.3 As the following two cases demonstrate, some patients with this condition may present without having experienced a single related symptom, while others
CASE SERIES
PATIENT 1 SM initially presented with complaint of moderate
rheumatoid arthritis, for which she had been taking methotrexate until her arthralgia had improved several months ago. However, since discontinuing that agent, her fatigue had been progressive.
Physical exam was notable for rheumatoid joint changes.
No splenomegaly was appreciated. She was found to have
a hemoglobin of 10.5 g/dL with anemia workup showing appropriately elevated erythropoietin level; and normal levels of folate, B12, haptoglobin, and lactate dehydrogenase. Serum protein electrophoresis was unremarkable.
room with severe fatigue, lightheadedness, and shortness
of breath. She exhibited tachycardia and general pallor. Hemoglobin measured 4.1 g/dL, WBC 9,500 cells/uL, and lymphocytes 77 percent (7,315 cells/uL). Peripheral smear demonstrated large lymphocytes with abundant cytoplasm and discharged with hemoglobin of 8.0g/dL.
It was believed that she had been affected by LGL leukemia for some time, but had been unintentionally treating this condition with methotrexate until her arthralgia had improved. Methotrexate was restarted for empiric treatment of LGL leukemia.
One month later, hemoglobin had improved to 9.9 g/dL, and a
LGL leukemia.
PATIENT 2 TD was referred to the Helen F. Graham Cancer Center and Research Institute (HFGCCRI) for
hysterectomy for cervical carcinoma-in-situ. Family history was
Patient was BRCA negative.
She had no pallor, lymphadenopathy, or splenomegaly on physical exam. Labs demonstrated hemoglobin of 13.5 g/dL and platelets of 282,000 cells/uL. On presentation, the white blood cell count and absolute neutrophil count of TD were 9,800 cell/ uL and 3,900 cells/uL, respectively.
demonstrated in Figure 1.
cytometry, CD4:CD8 ratio was inverted at 0.29:1; CD3+/CD8+/ CD57+ cells comprised 19.4 percent of total events; T-cell gene rearrangement testing was positive for a clonal rearrangement.
No treatment was initiated. Regular interval mammography was ordered. Tobacco cessation was recommended. The nature of her diagnosis and the need for active surveillance were explained to patient. Follow-up at six months with repeat CBC was planned.
DISCUSSION
LGL leukemia represents a subgroup of mature peripheral T-cell and (less commonly) natural killer cell neoplasms.
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