Ultrasonography for the Diagnosis and Follow-Up of Extramammary Paget Disease (EMPD): A Review.

Extramammary Paget disease (EMPD) is a rare skin tumor that manifests as poorly delimited lesions located mainly in genital area. Prognosis correlates with thickness and dermal invasion. The gold standard for diagnosis is the histopathological study of a biopsy. However, this technique is invasive and only shows a small area of the tumor. A good correlation has been reported between ultrasounds (US) and histopathologic finding. Moreover, a US examination has the advantages of wide availability, noninvasiveness, low cost, ease of use, and real-time scanning. We review the ultrasound signs described for this disease.

Multiorgan histiocytosis after B-cell acute lymphoblastic leukemia.

The association of multiorgan histiocytosis after acute lymphoblastic leukemias is very rare as most cases are localized forms of Langerhans cell histiocytosis (LCH). We report on an 18-year-old man diagnosed with B-cell acute lymphoblastic leukemia (B-ALL) with p16 deletion (9p21). He was treated with induction chemotherapy using the Spanish PETHEMA group protocol and achieved complete remission. Three months after the diagnosis of B-ALL, he developed a severe multiorgan histiocytosis that is clinically suggestive of LCH but lacked typical immunohistochemical features of LCH and indeterminate cell histiocytosis: CD1a was strongly positive, CD68 and S-100 protein were moderately positive, and langerin was negative. The drugs of the first-line treatment recommended for LCH had been part of the chemotherapy of B-ALL that the patient had received. Therefore, we prescribed the second-line treatment for LCH (cytarabine and 2'-chlorodeoxyadenosine), and he achieved partial remission. The patient died during the aplasia induced by the third cycle of chemotherapy from pneumonia. We could not demonstrate the transdifferentiation of tumoral lymphocytes into histiocytes, using p16 deletion (9p21) as a marker, because these cells did not share the mutation. Neither could we study immunoglobulin-H rearrangement as we had exhausted all the tissue samples. In the medical literature, there are a few reported cases of T-cell acute lymphoblastic leukemia followed by disseminated LCH and just 1 case of B-ALL followed by localized LCH affecting the bones. Therefore, our patient may be the first published case of B-ALL followed by histiocytosis, which had 2 singularities: it was multiorgan and the immunohistochemistry was not typical of LCH.

Assessing outcomes in liver disease patients: reliability and validity of the Spanish version of the Liver Disease Quality of Life Questionnaire (LDQOL 1.0).

OBJECTIVE: To assess the reliability and validity of a Spanish version of the LDQOL 1.0 (Liver Disease Quality of Life questionnaire). METHODS: Observational, cross-sectional study in Spanish patients awaiting liver transplantation (LT). Feasibility was assessed by analyzing administration times and missing responses. Ceiling and floor effects were calculated and reliability was tested by examining internal consistency (Cronbach's alpha). Convergent validity was tested by examining correlations between LDQOL disease-specific and Short Form health survey with 36 questions (SF-36) dimensions. Known groups' validity was tested by examining the LDQOL's capacity to discriminate between groups defined by etiology and Child-Turcotte-Pugh (CTP) scores. RESULTS: A total of 200 patients were included for analysis. Mean age (SD) was 52.6 (9.8) years and 73% of the sample were male. The most common indication for LT was liver cancer (34%). Mean (SD) time to complete the questionnaire was 35.8 minutes (21.2 minutes). Missing responses were highest on the dimensions of sexual functioning and symptoms of liver disease. Ceiling effects were over 20% on 7 of the LDQOL's 12 disease-specific scales. Cronbach's alpha coefficients were over 0.70 on all but 2 dimensions. Correlations between SF-36 and LDQOL disease-specific dimensions generally fulfilled the hypotheses, with 35 of the 40 highest and lowest correlations (87.5%) being in the expected direction. The LDQOL discriminated well between patients in CTP class A and C, and as hypothesized, hepatocarcinoma and alcoholic cirrhosis patients scored better on most dimensions than patients with hepatitis C virus or other etiologies. CONCLUSIONS: The Spanish version of the LDQOL 1.0 has shown satisfactory reliability and validity.