Nodular regenerative hyperplasia of the liver and cutaneous T-cell lymphoma: an unreported association.

Nodular regenerative hyperplasia of the liver -a type of noncirrhotic portal hypertension- is a rare condition of unknown etiopathogenesis that has been associated with multiple disorders, including diverse types of hematologic disease. We report the case of a 36-year-old female patient diagnosed with cutaneous T-cell lymphoma of the mycosis fungoides variety, staged as T2N0M0B0, where a transjugular liver biopsy demonstrated the presence of nodular regenerative hyperplasia with a hepatic venous pressure gradient of 15 mm Hg. The study was triggered by the incidental radiologic finding of hepatomegaly with indirect evidence of portal hypertension. We are not aware of any previous reports on the association of nodular regenerative hyperplasia with mycosis fungoides in the medical literature.

Disseminated juvenile xanthogranuloma associated with follicular lymphoma in an adult: successful treatment with chemotherapy and rituximab. A review of the literature.

Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis that typically affects children, but several cases have been reported in adults, some in connection with hematologic malignancies. We present the case of a 61-year-old woman with multiple xanthogranulomas who developed a follicular lymphoma after 4 years of follow-up. After 6 months of treatment with chemotherapy and rituximab, the cutaneous lesions disappeared and the patient achieved remission from lymphoma. We highlight this case because xanthogranuloma is a rare disorder that is difficult to diagnose in adults and also because this is the first report of an association between xanthogranuloma and follicular lymphoma. Excellent response was achieved with chemotherapy and rituximab. Finally, given the possible association between xanthogranulomas and hematologic diseases, these lesions may be a cutaneous manifestation of an occult malignancy.

Urticarial vasculitis: a retrospective study of 15 cases.

INTRODUCTION: Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis. OBJECTIVE: To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease. MATERIAL AND METHODS: We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years. RESULTS: Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus. CONCLUSIONS: Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease.

[Follow-up after surgical treatment of patients with familial adenomatous polyposis: results in a southern Spanish population]. / Seguimiento posquirúrgico de los pacientes con poliposis adenomatosa familiar: resultados en una población del sur de España.

OBJECTIVE: The study was to assess changes in the rectal mucosa and pouch in a series of patients with familial adenomatous polyposis (FAP) who underwent either subtotal colectomy and ileorectal anastomosis (IRA) or proctocolectomy and ileal pouch-anal anastomosis (IPAA), and to evaluate the suitability of the follow-up interval and postoperative treatment employed to prevent the development of cancer. METHOD: This study involved 28 patients with FAP who underwent IRA (n=20) or IPAA (n=8), and were followed endoscopically over a mean period of 7.47 years. The number and both macroscopic and histological features of polyps before and after surgery, the treatment, and complications were all analyzed. The suitability of the follow-up interval was assessed. RESULTS: None of the 26 patients who complied with follow-up developed rectal cancer. Two patients developed rectal cancer at 21 and 36 months after withdrawing from the protocol. Except in two cases in which surgery was indicated, patients who developed adenomas during follow-up were treated by endoscopic polypectomy. CONCLUSIONS: In our series, the failure to comply with follow-up examinations was associated with cancer development.

Role of SPECT-CT in sentinel lymph node biopsy in patients diagnosed with head and neck melanoma.

OBJECTIVE: Assess the role of SPECT-CT in sentinel lymph node (SLN) biopsy in the accurate anatomical location of the SNL in patients with cutaneous head and neck melanoma. MATERIAL AND METHODS: A retrospective study was conducted from February 2010 to June 2013 on 22 consecutive patients with a diagnosis of cutaneous head and neck melanoma (9 female, 13 male), with a mean age of 55 years old and who met the inclusion criteria for SLN biopsy. Patients underwent preoperative scanning after peri-scar injection of (99m)Tc-labeled-nanocolloid. Planar images of the injection-site, whole-body, and SPECT-CT scanning were acquired. RESULTS: Detection rate of SLN reached up to 91% (20/22 patients) by planar lymphoscintigraphy and 95.4% (21/22 patients) by SPECT-CT. SPECT-CT provided an accurate location of SLN in 14/22 patients, enabling to improve the surgical approach (clinical impact: 63.6%). SLN was positive for metastatic cells in 9.1% patients. CONCLUSION: SPECT-CT provides detailed anatomical SLN location and allows detecting a higher number of SLN than planar lymphoscintigraphy. Routine use of SPECT-CT is recommended in order to optimise the SLN detection and location in patients with head and neck melanoma.