A Case of Primary Renal Allograft Dysfunction Caused by Missed Monoclonal Disease.

We present an unusual etiology of primary renal allograft dysfunction attributed to myeloma cast nephropathy in a patient with no history of multiple myeloma before kidney transplant. The patient, a 54-year-old woman, had been on hemodialysis for 6 months before transplant for presumed diabetic nephropathy; she developed graft dysfunction immediately after transplant. Graft biopsy specimens were consistent with myeloma cast nephropathy, and she was treated with bortezomib, cyclophosphamide, and dexamethasone. She achieved a complete hematological response and regained excellent graft function 3 months after transplant. The patient then received autologous stem cell transplant 8 months after kidney transplant. To our knowledge, this is the second report of a successful graft outcome after chemotherapy and the first report treated with autologous stem cell transplantation after remission of monoclonal disease.

The Status of Spermatogenesis in Germ Cell Tumor Bearing Testis and Its Association with Metastatic Disease.

INTRODUCTION: We aimed to evaluate the status of spermatogenesis detected by histological examination of non-tumoral testicular tissues in tumor bearing testis and its association with advanced stage disease. PATIENTS AND METHODS: We retrospectively reviewed patients with testicular germ cell tumors (TGCTs) that undergone radical orchiectomy. All non-tumoral areas of the orchiectomy specimens were examined for the status of spermatogenesis. Patients were divided into two groups as localized (stage I) and metastatic (stage II-III) disease and analyzed separately for seminomatous (SGCT) and nonseminomatous germ cell tumors (NSGCT). RESULTS: Four hundred fifty-four patients were included in our final analysis. Of those, 195 patients had SGCT, and 259 patients had NSGCT. Three hundred and six patients had localized disease at the time of diagnosis. Median (Q1-Q3) age was 31 (26 - 38) years and 102 (22.5%) patients had normal spermatogenesis, 177 (39.0%) patients had hypospermatogenesis and 175 (38.5%) patients had no mature spermatozoa. On multivariate logistic regression analysis, embryonal carcinoma >50% (1.944, 95 %CI 1.054-3.585, P = .033) and spermatogenesis status (2.796 95% CI 1.251-6.250, P = .012 for hypospermatogenesis, and 3.907, 95% CI 1.692-9.021, P = .001 for absence of mature spermatozoa) were independently associated with metastatic NSGCT. However, there was not any variables significantly associated with metastatic SGCT on multivariate logistic regression analysis. CONCLUSION: Our study demonstrated that only 22.5% of patients with TGCTs had normal spermatogenesis in tumor bearing testis. Impaired spermatogenesis (hypospermatogenesis or no mature spermatozoa) and predominant embryonal carcinoma are associated with advanced stage NSGCT.

The role of treatment with plasma exchange therapy in two pediatric toxic epidermal necrolysis cases related to COVID-19.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening mucocutaneous reactions characterized by necrosis and detachment of the epidermis. Drugs and bacterial or viral infections are the most common causes of SJS/TEN. Although cases of SJS/TEN have been reported after hydroxychloroquine, vaccine (mRNA [Biontech], and inactivated vaccine [Sinovac]) administration and during the clinical course of active Coronavirus disease 2019 (COVID-19), limited data is indicating the COVID-19 disease as a triggering factor. Also, there are no pediatric cases of SJS/TEN associated with COVID-19 in the literature. Herein we reported two pediatric cases with a diagnosis of TEN related to COVID-19. Therapeutic plasma exchange therapy was applied to both of our patients. Although there are a few adult cases in the literature, our article is the first pediatric case report about patients diagnosed with TEN related to COVID-19 and successfully treated with plasma exchange.

A rare case of atrophic dermatofibroma with dermoscopic findings.

BACKGROUND: Dermatofibroma, also known as cutaneous benign fibrous histiocytoma, is a common skin tumour. AIM: The aim of this paper was to present a rare variant of dermatofibroma, atrophic dermatofibroma, emphasizing histopathological and dermoscopic features. PATIENTS/METHODS: A case of atrophic dermatofibroma in a female patient with the characteristic histopathological features and newly demonstrated dermoscopic findings is presented. RESULTS: A 54-year-old female presented with a depressed reddish lesion on the back showing histopathological findings of atrophic dermatofibroma. The dermoscopy of the lesion revealed a peripheral pigment network surrounding a pink-reddish colouration around a central whitish scar-like patch with white-yellow scales which was not an exact match with the description in the literature. CONCLUSION: Atrophic dermatofibroma is a rare variant that presents as an atrophic, depressed skin lesion which can easily be overlooked. Atrophic dermatofibroma should be considered in the differential diagnoses of atrophic, depressed lesions on the upper body of middle-aged women. The case of atrophic dermatofibroma presented here showed typical histopathologic findings with atypical dermoscopic features.

A case of dasatinib-induced focal segmental glomerulosclerosis in a patient with Philadelphia chromosome positive chronic myeloid leukemia.

Focal segmental glomerulosclerosis is a common glomerular histological lesion, which is usually characterised by non-nephrotic range proteinuria or nephrotic syndrome. It may be idiopathic or occurs secondarily to drugs, diabetes, obesity or HIV nephropathy and other infections. Dasatinib, a tyrosine kinase inhibitor that has been used for the treatment of Philadelphia chromosome-positive chronic myeloid leukemia, has a few renal adverse effects. Exceptional cases with non-nephrotic range proteinuria have been reported in relation with dasatinib. In this case, we report a patient with symptoms of nephrotic syndrome and nephrotic range proteinuria, who was diagnosed as focal segmental glomerulosclerosis by kidney biopsy after treated with dasatinib.

H syndrome with a novel homozygous SLC29A3 mutation in two sisters.

H syndrome (OMIM 602782) is a recently defined autosomal recessive genodermatosis. Cutaneous findings of H syndrome include hyperpigmentation, hypertrichosis, and induration, while hearing loss, heart anomalies, hepatomegaly, hypogonadism, hyperglycemia (diabetes mellitus), low height (short stature), hallux valgus (flexion contractures), and hematological abnormalities are the extracutaneous abnormalities. We report a novel homozygous missense mutation, c.416T > C p.(Leu139Pro), in the SLC29A3 (NM_001174098.1) gene in two sisters with H syndrome presenting with different phenotypes.