Cases reported "Mycosis Fungoides"

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1/4. Cases from the Osler Medical Service at Johns Hopkins University.

    PRESENTING FEATURES: An 85-year-old black woman presented to the Osler Medical Service complaining of a pruritic, erythematous scaly rash that was on her right thigh and abdomen and that had been worsening over the prior 3 months. She also complained of increasing fatigue, decreased exercise tolerance, and a 5-lbs weight loss. There was no orthopnea, paroxysmal nocturnal dyspnea, fevers, chills, or night sweats. She denied recent travel and exposures to or contact with ill people. Her past medical history was unremarkable. There was no history of eczema, atopy, or dermatologic conditions. Her only medication was a baby aspirin taken daily. On physical examination, she was afebrile, her blood pressure was 110/72 mm Hg, and her pulse was 82 beats per minute with a room air oxygen saturation 98%. She was mildly obese but in no apparent distress. She had 1-cm anterior cervical lymphadenopathy bilaterally and a 1-cm left axillary lymph node. Cardiovascular and chest examination was unremarkable. Her abdomen was soft and nontender, with a faint, erythematous rash that was mildly scaly but nontender in her abdominal skin fold. Her right thigh showed a large erythematous area, approximately 15 x 20 cm, which was scaly with multiple areas of discoloration (Figure 1). There was no palpable mass. She had slight edema in her right leg. Her left thigh was normal. neurologic examination was nonfocal. Laboratory studies were notable for a white blood cell count of 17,000 cells/microL, with a differential of 46% lymphocytes. The absolute lymphocyte count was 8000 cells/microL. hematocrit was 28%. platelet count was normal. A comprehensive metabolic panel was normal. A peripheral blood smear (Figure 2) showed numerous atypical lymphocytes with cerebriform nuclei. Peripheral blood flow cytometry showed the presence of a clonal population of T cells that expressed CD4 and CD5 but showed a loss of CD7. What is the diagnosis?
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2/4. Coexistent B-cell lymphoma and cutaneous T-cell lymphoma.

    A 78-year-old man with a history of mycosis fungoides was referred for evaluation of a right adrenal mass. A physical examination showed the left cervical lymph node to be palpable, which was later shown to be caused by a diffuse large B-cell lymphoma. The patient was diagnosed with concurrent mycosis fungoides and a diffuse large B-cell lymphoma. Three courses of chemotherapy were performed, however, the patient died of advanced disease. autopsy findings showed that the right adrenal and soft tissue masses had an identical B-cell origin. Although the exact mechanism remains unclear, the pathogenesis of this rare association is discussed.
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3/4. Remission in skin infiltrate of a patient with mycosis fungoides treated with cyclosporine.

    A 57-year-old woman with mycosis fungoides that had failed to respond to cytotoxic chemotherapy was treated with cyclosporine. mycosis fungoides and sezary syndrome are disorders of helper T cells. cyclosporine is a fungal endecapeptide of novel chemical structure that causes preferential inhibition of T helper cells. Because of this in vitro inhibition of T helper cells, we used cyclosporine to treat a patient who had mycosis fungoides that was refractory to cytotoxic combination chemotherapy. With cyclosporine administered initially as an intravenous infusion and orally after 10 days, there was immediate improvement in the patient's symptoms. This subjective improvement was accompanied by a decrease in her skin infiltration, noted on physical examination and microscopically. Despite continued administration of the cyclosporine, symptoms recurred after 3 1/2 months of therapy.
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4/4. Exposure to chemicals, physical agents, and biologic agents in mycosis fungoides and the sezary syndrome.

    The history of occupational, environmental, and/or iatrogenic exposure to potential carcinogenic agents was obtained at the time of onset of skin disease in 43 of 44 patients with cutaneous T-cell lymphoma (mycosis fungoides and the sezary syndrome) entering a National Cancer Institute therapeutic trial. A history of multiple exposures to these agents was common, the two most frequent being chemicals (91% of patients) and drugs (86%). Mean duration of expsosure was 13 years for chemicals and 18 years for drugs. The most common chemicals were air pollutants (39%), pesticides (36%), solvents and vapors (30%), and detergents and disinfectants (14%). Increased severity of disease was seen with increaed duration of chemical exposure in stage V cutaneous T-cell lymphomas only. The most frequent drugs besides tobacco (86%) were analgesics (20%), tranquilizers (18%), and thiazides (14%). Second cancers occurred in four patients, including two renal cell carcinomas, and a family history of cancer was present in 11. Fourteen of 22 patients questioned had recurrent herpes simplex. patients with chronic skin disease who have long-term exposure to combinations of chemicals, physical agents, and biologic agents, are heavy smokers, or have recurrent herpes simplex appear to be prime candidates for developing mycosis fungoides or the sezary syndrome.
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