A 76-year-old patient with a history of COPD and coronary heart disease.
At the beginning of January 2013 she began with pruritus, without skin lesions, some episodes of febrile syndrome, fatigue and decreased appetite, which led to multiple consultations.
In May of the same year, progressive cutaneous involvement was added, which ended in erythrodermia.
Physical examination revealed marked xerosis, grating signs, intense generalized erythema and palpable cervical and inguinal lymphadenopathy.
In June 2013 a skin biopsy was performed, whose initial report was interpreted as an adverse drug reaction; however, the blood count showed 30% of pathological lymphocytes, so when the biopsy reassessed the hypercholesterolemia large lymphocytes were reported.
Flow cytometry (FCM) in peripheral blood showed 11% Sézary cells.
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A CT scan of the neck, chest, abdomen and pelvis was requested, highlighting small adenopathies in both jugular chains, posterior triangles of the neck, supraclavicular and inguinal proliferative syndrome.
Bone marrow biopsy was negative for lymphoma; HTLV-1 serology, Epstein-infection and cytomegalovirus were negative.
Stage IIIb SS was diagnosed (T4-N1-M0-B1), entered the GES system (explicit health care guarantees) in July 2013 and started treatment with PUVA chemotherapy (12 sessions) associated with bamboo.
Due to the poor response, she was referred to our center for evaluation and management.
On admission, the patient presented erythrodermia, grating signs, xerosis and lymphadenopathy.
In addition, he had a 7 mm diameter crateiform nodule in the right nasal cavity, clinically complete on October 96 with spinocellular carcinoma. He had a new blood count with 50% of pathological lymphocytes and an ultrasonographic (LDH 2 sessions).
Peripheral unipuncture was performed in each session, an approximate treatment volume of 260 ml was extracted, and 4.4 ml of methoxalen and heparin were used as anticoagulant, with an average duration of 3 h.
Pruritus was managed with anti H1 (hydroxycin) and topical emollients.
The patient responded well, achieving 100% control of pruritus, with complete regression of skin lesions.
A new post-treatment biopsy showing epidermal necrolysis with superficial laxa fibrosis and atypical lymphocytes was performed.
MFC showed a decrease in Sézary cells from 11% at baseline to 0.7% at sessions of ECF.
Blood cell counts decreased from 50% to 0% in 4 months.
In March 2014, a biopsy of a lesion of the right nasal wing showed a spinocellular carcinoma, treated with electron beam for 6 weeks with excellent response.
Currently, it has 11 months of remission.
FMC-Use in October 2014 showed no Sézary cells.
She remains on treatment with chlorambucil and prednisone.
