A 42-year-old male presents with two-year history of a diffusely erythematous scaly rash over the
trunk and extremities. He describes the rash as so intensely pruritic that he bathes several times a
day in cold water to relieve the burning. On physical examination, he has multiple erythrodermic
patches over his face and body. Abdomen is soft, non-tender, with no organomegaly. There is no
lymphadenopathy. His vitals are within normal limits. A complete blood count is as follows:
Hb: 14 g/dL (NR for males: 13.5-17.5)
WBC: 23,000/mm3 (NR 4000-11,000)
- Polymorphonuclear cells: ~10%
- Lymphocytes: ~90%
Peripheral smear examination reveals large lymphoid cells with prominent nuclear indentations and
grooving. Bone marrow examination is unremarkable. Transmission electron microscopy (TEM)
reveals characteristic cells with cerebriform nuclei. Which of the following is the most likely
diagnosis?
- A) Sezary syndrome
- B) Mycosis fungoides
- C) HIV/AIDS
- D) Kaposi sarcoma
- E) Lichen planus
0 voters
EXP:
Sezary syndrome is a form of low-grade cutaneous T cell non-Hodgkin lymphoma with leukemic
extension to the peripheral blood; the purely cutaneous form of the disease is known as mycosis
fungoides.1 In other words, the syndrome is understood to be the advancing leukemic phase of
mycosis fungoides.2
Sezary syndrome is characterized by the presence of “fiery red” erythroderma, generalized
lymphadenopathy (in the early stages), and the presence of Sezary cells in the skin, lymph nodes,
and peripheral blood.3, 4 Sezary cells are described as “cerebriform” for their prominent nuclear
indentations and grooving.5
The development of mycosis fungoides and Sezary syndrome may be caused by human T cell
lymphotropic virus (HTLV).6, 7
• “Here we report the presence of HTLV pol and/or tax proviral sequences in 46 out of 50 (92%)
of the patients tested. In addition, five of the patients, who lacked antibodies to HTLV-I/II
structural proteins, were found to be seropositive for tax. It thus seems reasonable to conclude
that MF/Sézary syndrome is an HTLV-associated disease and that lack of an immune response
does not preclude infection with this type of virus.”6
• “The human T-cell lymphotropic virus type I (HTLV-I) is a retrovirus that causes adult T-cell
leukemia–lymphoma, which can also involve the skin and be difficult to distinguish from a
primary cutaneous T-cell lymphoma.”8
The USMLE also wants you to know that HTLV is endemic to Japan, with a contemporaneous
increased prevalence of adult T cell leukemia and lymphoma.9 HTLV is also associated with IV
drug use.10 Their contraction via IV drug use should not be a surprise as HTLV and HIV are both
retroviridae, and HIV is frequently acquired via IV drug use. There have also been studies in the
United States demonstrating the relationship of IV drug use to HTLV and HIV