We report a case of a 3-month-old female baby showing clinical and hematological signs compatible with Kawasaki disease. A few days after the beginning of the specific therapy, the young patient developed asymptomatic erythematous plaques appearing on the trunk. The histology disclosed a psoriasic pattern. To the best of our knowledge, we describe the youngest baby in the published work developing a psoriasis during the course of Kawasaki disease.
Hematopoietic stem and progenitor cells (HSPCs) are multipotent stem cells that have been harnessed as a curative therapy for patients with hematological malignancies. Notably, the discovery that HSPCs are endowed with immunoregulatory properties suggests that HSPC-based therapeutic approaches may be used to treat autoimmune diseases. Indeed, infusion with HSPCs has shown promising results in the treatment of type 1 diabetes (T1D) and remains the only “experimental therapy” that has achieved a satisfactory rate of remission (nearly 60%) in T1D. Patients with newly diagnosed T1D have been successfully reverted to normoglycemia by administration of autologous HSPCs in association with a non-myeloablative immunosuppressive regimen. However, this approach is hampered by a high incidence of adverse effects linked to immunosuppression. Herein, we report that while the use of autologous HSPCs is capable of improving C-peptide production in patients with T1D, ex vivo modulation of HSPCs with prostaglandins (PGs) increases their immunoregulatory properties by upregulating expression of the immune checkpoint-signaling molecule PD-L1. Surprisingly, CXCR4 was upregulated as well, which could enhance HSPC trafficking toward the inflamed pancreatic zone. When tested in murine and human in vitro autoimmune assays, PG-modulated HSPCs were shown to abrogate the autoreactive T cell response. The use of PG-modulated HSPCs may thus provide an attractive and novel treatment of autoimmune diabetes.
ObjectiveThe objective of this study was to profile patients who undergo defecography, by age and gender, as well as to describe the main imaging and diagnostic findings in this population.Materials and MethodsThis was a retrospective, descriptive study of 39 patients, conducted between January 2012 and February 2014. The patients were evaluated in terms of age, gender, and diagnosis. They were stratified by age, and continuous variables are expressed as mean ± standard deviation. All possible quantitative defecography variables were evaluated, including rectal evacuation, perineal descent, and measures of the anal canal.ResultsThe majority (95%) of the patients were female. Patient ages ranged from 18 to 82 years (mean age, 52 ± 13 years): 10 patients were under 40 years of age; 18 were between 40 and 60 years of age; and 11 were over 60 years of age. All 39 of the patients evaluated had abnormal radiological findings. The most prevalent diagnoses were rectocele (in 77%) and enterocele (in 38%). Less prevalent diagnoses were vaginal prolapse, uterine prolapse, and Meckel's diverticulum (in 2%, for all).ConclusionAlthough defecography is performed more often in women, both genders can benefit from the test. Defecography can be performed in order to detect complex disorders such as uterine and rectal prolapse, as well as to detect basic clinical conditions such as rectocele or enterocele.
Background Primary bone lymphoma (PBL) has been described as a malignant lymphoid bone infiltration, with or without cortical invasion and/or soft tissue extension, without visceral involvement nor distantlymph nodes. PBL constitutes 2% of all bone tumors and 5% of extranodal lymphomas. Sites commonly affected are the long bones and only 1-2% has primary vertebral locations. The common symptoms are inflammatory pain, palpable mass, pathologic fractures or spinal cord compression syndrome. In 50% of cases was B symptoms (fever, weight loss and night sweats) before and the majority are monostotic (80%). Histopathologically, the majority of cases has been diffuse large B-cell non-Hodgkin lymphoma but has been cases of follicular B-cell lymphoma. The usual treatment is chemotherapy with rituximab-CHOP regimen followed by radiotherapy if there is residual diseaseor spinal cord compression. Patients have 5 and 10 years overall survival of 60% and 45% respectively. Objectives report 5 cases of PBL in a county hospital. Methods 5 patients with PBL were collected; they were 2.5% of all lymphomas diagnosed in our hospital during 1995-2011. We analyzed the clinical features,laboratory and radiologic findings, treatment and follow-up of these patients. Results 5 patients were diagnosed(3 men and 2 women), median age was 54 years (35-73). Four had inflammatory pain and one palpable mass. No patient had B symptoms. Analysis only showed alterations in acute phase reactants in two patients and the study of tumor markers, LDH levels and serum protein were normal in all patients. Plain radiographs showed only changes (vertebral fractures) in patient 3. Malignant lesion was suspected by MRI study (heterogeneous hypointense lesion on T1 and hyperintense on T2) and in patient 5 by radionucleotide 99mTc bone scanning. CT scan not showed visceral involvement or lymphadenopathy in any patient. Only in one case (patient 3) bone biopsy showed bone marrow infiltration. The injury was monostotic in two cases and polyostotic in three. The histological diagnosis was confirmed by bone biopsy (WHO classification). Four were diffuse large B-cell lymphoma and one follicular B. Stage was assigned according to Ann-Arbor staging system, IE in two patients, IIE in two and IVE in one. Treatment consisted of radiation therapy to the patient 4 (follicular lymphoma) and chemotherapy with rituximab-CHOP regimen in three cases. Patient 5 was treated with chemotherapy according to CHOP regimen and required a second course of chemotherapy and autologous peripheral blood transplantation (ASCT) for cutaneous recurrence. Patients 4 and 5 obtained complete remission after treatment, while patient 4 died four years later from a hepatocellular carcinoma. To remark,in three patients extension study found presence of thyroid disease (two goiters and one nodule without malignancy). Conclusions ThePBL is a rare bone tumor and especially if there is primary vertebral involvement. Symptoms and radiological findings are variable and nonspecific. Primary bone lesion b...
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