AMA Publishing Group: Hematology Topic Collection

Primary Cutaneous CD4 + Small- to Medium-Sized Pleomorphic T-cell Lymphoma Temporary Remission by Oral Doxycycline Primary Cutaneous CD4 + Pleomorphic T-cell Lymphoma

Toberer F, Hartschuh W, Hadaschik E. — Wed, 12 Jun 2013 00:00:00 GMT

Importance

In the recent World Health Organization–European Organisation for Research and Treatment of Cancer classification, primary cutaneous CD4⁺ small- to medium-sized pleomorphic T-cell lymphoma is listed as a provisional entity that is histopathologically characterized by pleomorphic CD3⁺/CD4⁺/CD8⁻/CD30⁻ T lymphocytes. Clinically, it is characterized by solitary tumors mostly affecting the head and neck area and by an indolent clinical course with an estimated 5-year survival of about 60% to 80%. Currently, therapeutic options include topical or systemic treatment with glucocorticoids, local excision of solitary lesions, radiotherapy, and chemotherapy (eg, cyclophosphamide) in cases of aggressive clinical behavior or systemic disease.

Observations

We present the case of a 21-year-old female patient with a 5-year history of a solitary, slowly growing tumor of the right cheek. Histopathologic findings revealed a primary cutaneous CD4⁺ small- to medium-sized pleomorphic T-cell lymphoma with an admixture of numerous CD20⁺ B cells representing almost half of the infiltrate. In this patient we achieved a temporary (13 months) complete remission of the lymphoma by oral treatment with doxycycline monohydrate, 200 mg per day.

Conclusions and Relevance

Doxycycline is a relatively nontoxic and well-tolerated oral agent and should be considered as a therapeutic option in primary cutaneous CD4⁺ small- to medium-sized pleomorphic T-cell lymphoma, especially in cases with a high percentage of B lymphocytes and no signs of systemic disease.

Rapidly Enlarging Nodule on the Scalp—Diagnosis

Wed, 12 Jun 2013 00:00:00 GMT

Image of the Month—Quiz Case

Mueller K, Eckford J, Watson RW. — Wed, 12 Jun 2013 00:00:00 GMT

A 64-year-old woman presented to the emergency department with a 1-day temperature of 38.9°C (102°F). The patient had some accompanying cough, nausea, and arthralgias but denied having dyspnea, emesis, and chest or abdominal pain. She had a complicated medical history that was significant for non-Hodgkin lymphoma, chronic diarrhea, myelodysplastic syndrome treated with chemotherapy, and Behçet syndrome treated with prednisone acetate (6 mg daily). Her surgical history was significant for a previous right-sided hemicolectomy due to recurrent right-sided diverticulitis. On examination, she was alert and awake with appropriate mental status. Her vital signs were as follows: blood pressure of 150/59 mm Hg, heart rate of 127 beats per minute, respiratory rate of 20 breaths per minute, temperature of 38.5°C (101.3°F) (oral), and an oxygen saturation as measured by pulse oximetry of 99% in room air. The physical examination was unremarkable. Significant laboratory data included a white blood cell count of 2700/μL (reference range, 4800-10 800/μL; to convert to ×10⁹ per liter, multiply by 0.001), a lactic acid level of 19.8 mg/dL (to convert to millimoles per liter, multiply by 0.111), and a platelet count of 72×10³/μL (to convert to ×10⁹ per liter, multiply by 1.0). Urinalysis showed trace blood, positive nitrite, +1 leukocyte esterase, and a white blood cell count of 2000/μL to 5000/μL. A urine culture came back positive for Klebsiella pneumonia, and a blood culture came back positive for Enterobacter sakazakii (now know as Cronobacter sakazakii). There was concern about the source of the bacteremia, so an infectious disease consult was ordered, and a computed tomographic scan of the abdomen/pelvis with contrast was performed. Figure 1 shows a coronal, reformatted image of the scan. The patient was scheduled for surgery, and the findings are shown in Figure 2.