CME examination Identification No. 890-103
Instructions for Category 1 CME credit appear in the front advertising section. See last page of Contents for page number.
Questions 1-33, Wieselthier JS, Koh HK. JAM ACAD DERMATOL 1990;22:381-401.
Directions for questions 1-16: Give the single bestresponse.
1. Which of the following clinical features of Sezarysyndrome is present in virtually all cases?a. Adenopathyb. Hepatomegalyc. Alopeciad. Keratoderma and/or onychodystrophye. Intense pruritus
2. All of the following have been reported in associationwith Sezary syndrome, excepta. edemab. hepatomegalyc. onychodystrophyd. alopecia mucinosae. Pautrier's microabscesses
3. Currently available data suggest that the most common cause of erythroderma isa. drug eruptionb. Sezary syndromec. atopic dermatitisd. contact dermatitise. psoriasis
4. In the various staging systems for human cutaneousT cell lymphomas (CTCL), Sezary syndrome is generally classified as being in stagea. Ib. IIc. IIId. Ve. None of the above is correct
5. Median survival for patients with Sezary syndromeis approximatelya. 6 to 9 monthsb. 6 months to 1 yearc. 1 to 5 yearsd. 5 to 8 yearse. 8 to 15 years
6. A recently developed test for identifying the clonalnature of Sezary syndrome is detection ofgene rearrangements in thea. IX subunit of the B cell receptorb. (3 subunit of the B cell receptorc. IX subunit of the T cell receptord. (3 subunit of the T cell receptore. 0 subunit of the T cell receptor
7. Which of the following drugs is concentrated, or hasa particularly enhanced effectiveness, in skin?
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a. Methotrexateb. Bleomycinc. Adriamycind.Azaribinee. Prednimustine
8. Which of the following is an inhibitor of adenosinedeaminase?a. Bleomycinb. Prednimustinec. Adriamycind. Deoxycoformycine. Tilorone hydrochloride
9. Which of the following diseases is associated with aprimary deficiency of adenosine deaminase?a. DiGeorge syndromeb. Di Guglielmo's diseasec. Combined immunodeficiency syndromed. S6zary syndromee. Acquired immunodeficiency syndrome (AIDS)
10. A possible association has been proposed betweenSezary syndrome and which ofthe following viruses?a. Herpes simplex, type 1b. Cytomegalovirusc. Human T-cell leukemia virus 1 (HTLV-I)d. Human T-cell leukemia virus 3 (HTLV-III)e. Human T-cell leukemia virus 5 (HTLV-V)
11. Leukovorin is used in combination witha. adriamycin, to prevent its clearance via the kid-
neys and thus to enhance its effectivenessb. azaribine, to enhance its effectivenessc. methotrexate, to ameliorate its toxicityd. methotrexate, to enhance its effectivenesse. methotrexate, as a subsequent ("rescue") applica
tion, to ameliorate its toxicity, thereby allowinghigher doses to be given
12. The most common side effect of interferon (interferon alfa-2a) therapy for Sezary syndrome isa. fiulike illness with pyrexia (fever)b. clinically apparent hepatotoxicityc. leukopeniad. reversible nephrotic syndromee. irreversible nephrotic syndrome
13. Of the following, the most important side effects ofcyclosporine therapy area. leukopenia and bone marrow suppressionb. granulocytopenia and infectionc. hepatotoxicity and nephrotoxicity
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d. neurotoxicity and temporary dementiae. colitis and hemorrhagic cystitis
14. Approximately what proportion ofpatients receivingtopical mechlorethamine for S6zary syndrome havea delayed hypersensitivity reaction to the drug?a.l%b. 8%c.15%d.33%e. More than 50%
15. Interferon has been noted to induce all of the following in cancer cells, excepta. decreased total cellular levels of transcription
(RNA synthesis)b. increased activities of certain DNA repair en-
zymesc. restoration of contact inhibition in vitrod. tumorostasis in vivoe. alterations in differentiation and metastasis in vivo
16. Cross-links between strands of the DNA moleculeare an important lesion in DNA produced by treatment witha. bleomycinb. azaribinec. methotrexated. adriamycine. psoralen with ultraviolet A (PUVA)
Directionsfor questions 17-21: For each numbereditem, choose the one lettered item corresponding tothe author(s) who first reported it. Each lettereditem may be usedonce, more than once, or not at all.
a. S6zary and Bouvrainb. Besnier and Hallopeauc. Alibertd. Taswell and Winkelmanne. Edelson
17. Coined the term Sezary syndrome18. Coined the term mycosis fungoides19. Coined the term cutaneous T eel/lymphoma20. First described the erythrodermic variant of cutane
ous T cell lymphoma21. Described a patient with the clinical constellation
of erythroderma, intense pruritus, adenopathy, andcirculating abnormal "monster" hyperconvolutedmononuclear cells in the peripheral blood
Directionsfor questions 22-25: For each numbereddrug, choose the one lettered toxic side effect that itisparticularly prone to produce. Each lettered itemmay be used once, more than once, or not at all.
a. Nausea and vomitingb. Hemorrhagic cystitis
CME examination 403
c. Cardiomyopathyd. Pulmonary fibrosise. Peripheral neuropathy
22. Adriamycin23. Bleomycin24. Cyclophosphamide25. Vincristine
Directions for questions 26 and 27: For eachnumbered item choose the appropriate lettereditem.
a. Leu-8b. Leu-9c. Bothd. Neither
26. Major T cell marker27. Consistently deficient in S6zary syndrome
Directionsfor questions 28-31: For each numbereditem choose the appropriate lettered item.
a. Acyclovirb. Tilorone hydrochloridec. Bothd. Neither
28. Antiviral agent29. Inhibits viral DNA synthesis30. Induces interferon31. Reported to have a beneficial effect on S6zary
syndrome in isolated cases
Directionsfor questions 32 and 33: Indicate correctanswers. All, some, or none of the choices may becorrect.32. Circulating Sezary cells have been reported in pa
tients witha. B cell lymphomab. vasculitisc. lichen planusd. atopic dermatitise. solar keratoses
33. Which of the following has/have been proved to beconsistently effective, in multicenter randomizedclinical studies, for S6zary syndrome?a. Isotretinoin and arotinoid-ethylester (Ro 13
6298)b. Electron beam irradiation with doxorubicin, cy
clophosphamidec. Electron beam irradiation with topical mechlor
ethamined. Bleomycin, cyclophosphamide, prednisone plus
etretinatee. Chlorambucil, prednisone plus radiation therapy