A 36-year-old woman was referred to our consultation due to pathology and hematology for the study of an incidental radiological hepatomegaly.
Since approximately three years before, the patient had skin lesions with eccematous appearance, plaques, some of them pigmented, erythematous-desquamative, not very pruritic and circular upper limbs.
She had undergone two previous cutaneous biopsies, with nonspecific results suggested the presence of pityriasis with parakeratosis modalities, minimal superficial perivascular lymphocytic infiltratecc -with the presence of eosinophils- and mild topical pinkish hyperplasia.
Four months before our first visit, a mycosis fungoides-type CTCL was finally diagnosed.
On this occasion the skin biopsy showed a dense superficial dermal inflammatory lymphocytic infiltrate with minimal exocytosis in epidermis but manifests in hair bulb; the immunophenotype of CD3-30 % was predominantly mitotic component.
Amplification of the JV segment of the hypervariable region of the TCR-γ gene (T cell receptor-gamma) was performed by means of a dressing with specific primers and successive fragment analysis (Grenada Biosystem, Spain).
The analyzed sample contained a tumor cellularity greater than 70 % and a DNA quantity of 21.2 ng/μl.
A specific monoclonal T-line rearrangement was confirmed: the analyzed material showed a fragment of amplification of the size of 156 base pairs.
At the time of diagnostic confirmation of CTCL, the patient was 26 weeks pregnant, which limited the therapeutic possibilities.
As part of the staging, an i.v. contrast-enhanced CT scan was programmed, which also detected a vesicular rash until delivery, which detected a homogeneous hepatomegaly without space-occupying lesions as the only significant alteration.
Stage at diagnosis was established as T2 (affected more than 10 % of the body surface), N0, M0, non-normal lymphocyte count in peripheral blood smears and CD8 lymphocytes, or changes in peripheral blood smears or other organs).
In our first visit the patient had completed a cycle of 24 sessions of phototherapy with ultraviolet B radiation (cumulative dose of 19,804 MJ), with significant improvement.
On the other hand, the patient was asymptomatic, there were no palpable abdominal masses or adenopathies and no stigmata of chronic liver disease on the general inspection.
Body mass index was 20.6 kg/m2.
The blood count was normal in all three series and the usual parameters showed rigorously preserved liver function.
Liver enzymes were normal.
Serology for hepatitis B and C virus were negative as well as autoantibodies associated with liver disease.
There was no iron overload or alterations in the proteinogram or immunoelectrophoresis.
Three months after stopping phototherapy, the patient had a severe recurrence of the skin lesions, so she was treated with beta-blockers (150 mg/m2: 225 mg daily, adjusted for her body surface area).
Simultaneously, i.v. contrast-enhanced CT was repeated, in which radiological data of HTP (increased caliber, pattern of peripheral hypertension and persistent "hepatic portal hypertension" persisted.
In this situation a transjugular liver biopsy was performed with simultaneous measurement of pressures enclaved (25 mm Hg) and free (10 mm Hg) in suprahepatics: hepatic venous pressure gradient (HVPG).
No sedation was performed for this procedure.
Five fragments of liver tissue of oscillating sizes between 5 and 10 mm were histologically analyzed: there was distortion of architecture with alteration of the relationship of central veins with portal tracts and absence of fibrosis.
No gastrointestinal varices or other manifestations of PH were documented on oral endoscopy.
Abdominal ultrasound showed no evidence of steatosis.
Hepatic stiffness as measured by the loop transition (ET) showed a result of 6.1 kilopascales (kPa) with an interquartile range of 0.8 kPa.
The urinary excretion of Na/K obtained at baseline was 132/141 mmol/24 hours.
No alterations of mention were found in a complete hypercoagulation study that included determinations of antithrombin III, proteins C and S, homophobicin, antiphosphobic antibody and the Leiden mutation factor.
Six months have passed since the diagnosis of NRH without complications.
