Aims
The current WHO classification of melanocytic tumours excludes neoplasms showing BRAF or NRAS mutations from the Spitz category. This study aimed to review and reclassify atypical melanocytic ...tumours with spitzoid morphological features diagnosed between 2009 and 2021 in our hospital after expanding the molecular profile, including BRAF and NRAS mutations in all cases.
Methods and results
A total of 71 neoplasms showing spitzoid features (Spitz‐like) and atypia were included. The risk of progression of tumours was first studied by integrating the morphology, immunohistochemistry (p16, Ki67, HMB45 and PRAME) and fluorescence in‐situ hybridisation (FISH) results (melanoma multiprobe and 9p21). In a second step, after expanding the molecular study, including BRAF and NRAS mutational status, the neoplasms were finally classified into four subgroups: atypical Spitz tumour (AST, n = 45); BRAF‐mutated naevus/low‐grade melanocytoma with spitzoid morphology (BAMS, n = 2); Spitz melanoma (SM, n = 14); and BRAF or NRAS mutated melanoma with spitzoid features (MSF, n = 10). Follow‐up of patients revealed uneventful results for AST and BAMS. Only one SM presented lymph node metastasis after 134 months. Conversely, patients with MSF showed an unfavourable outcome: three developed lymph node metastases after a mean time of 22 months, with one patient presenting distant metastasis and dying of the disease 64 months from diagnosis. The progression‐free survival showed significant differences between the four groups of spitzoid tumours (P < 0.001) and between both melanoma subtypes (P = 0.012).
Conclusions
The classification and prognostication of atypical neoplasms with spitzoid features requires the integration of histomorphology with the molecular investigation of tumours, which should include BRAF and NRAS mutational status.
The classification and prognostication of atypical neoplasms with spitzoid features requires the integration of histomorphology with the molecular investigation of tumours, which should include BRAF and NRAS mutational status.
Summary
Carfilzomib has been associated with the development of thrombotic microangiopathy (TMA) in relapsed/refractory multiple myeloma patients, a severe disease with no currently available ...aetiological treatment. We evaluated the potential role of terminal complement pathway in four patients with carfilzomib‐induced TMA. Membrane attack complex (C5b‐9) deposition on endothelial cells in culture exposed to plasma from patients during the acute phase of the disease suggests complement overactivation as a mechanism of potential endothelial damage in three out of four patients. If confirmed in larger cohorts, C5b‐9 evaluation will allow early identification of patients who could benefit from complement blockade and treatment monitoring.
Background
Primary cutaneous follicular center‐cell lymphoma (PCFCL) is one of the most common types of cutaneous B‐cell lymphoma. Differences in immunohistochemical expression of BCL2 and CD10 ...antigens along with the presence of t(14:18) translocation in neoplastic cells have been postulated as relevant clues in differentiating PCFCL from cutaneous lesions secondary to a systemic follicular lymphoma (SCFL). The aim of this study is to evaluate the significance and usefulness of these parameters in a large series of patients.
Methods
Patients with PCFCL and SCFL diagnosed at three university hospitals in Barcelona, from 2000 to 2015 were reviewed. Clinical, histopathological, immunophenotypical, genetic, and outcome parameters were analyzed.
Results
Eighty‐one cases (59 PCFCL and 22 SCFL) were included. There were no significant differences between PCFCL and SCFL cases regarding clinical presentation, site of involvement, or predominant type of skin lesions. Most patients in both groups showed positivity for BCL2 and CD10, but strong expression of BCL2 and CD10 was associated with SCFL cases. Although more frequent in SCFL, a small proportion of PCFCL cases also showed the t(14:18) on FISH analysis.
Conclusion
The intensity of BCL2 expression was found to be the single most valuable clue in differentiating PCFCL from SCFL cases on histopathological grounds.
Introduction
HLA sensitization is a growing problem in children awaiting kidney transplantation. In some cases, finding an immunologically compatible donor entails contemplating the option of an ABO ...incompatible transplant or paired transplant.
Methods
Patient with genetic nephrotic syndrome and progressive chronic kidney disease, with a previous thrombosis of a first kidney transplant, resulting hypersensitized and remaining for a long‐time on hemodialysis. Despite a desensitization strategy, family members were incompatible and deceased donation options must be ruled out due to the presentation of donor‐specific antibodies (DSA). After 4 years, the possibility arises to perform a kidney paired transplant with a 62‐year‐old woman with an incompatible blood group. Although the current cytotoxicity‐ and cell‐based crossmatches were negative, history of DSA were recorded.
Results
An intensive ABO and HLA desensitization protocol was performed in order to combat the isohemagglutinin antibodies and on the memory‐HLA, based on rituximab, apheresis sessions, and immunoglobulins. Despite the donor being older in terms of pediatric transplantation, the donor‐recipient weight difference, and immunological risk, the transplant was completed successfully. Maintenance of titration of up to 1/2 was confirmed after 3 weeks post‐transplant (IgM and IgG). Kidney biopsy at 2 weeks and 6 months without signs of rejection. The patient is currently 12 months post‐transplant and has not presented any signs of transplant rejection and has proper renal function.
Conclusions
Kidney paired transplantation is an excellent solution for hypersensitized children, and ABO incompatibility can be considered to increase their options to find a good donor, without thereby obtaining worse results.
The dialysis‐based definition of Delayed Graft Function (dDGF) is not necessarily objective as it depends on the individual physician’s decision. The functional definition of DGF (fDGF, the failure ...of serum creatinine to decrease by at least 10% daily on 3 consecutive days during the first week post‐transplant), may be more sensitive to reflect recovery after the ischemia‐reperfusion injury. We retrospectively analyzed both definitions in 253 deceased donor kidney transplant recipients for predicting death‐censored graft failure as primary outcome, using eGFR < 25 ml/min/1.73 m2 as a surrogate end‐point for graft failure. Secondary outcome was a composite outcome that included graft failure as above and also patient’s death. Median follow‐up was 3.22 2.38–4.21 years. Seventy‐nine patients developed dDGF (31.2%) and 127 developed fDGF (50.2%). Sixty‐three patients fulfilled criteria for both definitions (24.9%). At multivariable analysis, the two definitions were significantly associated with the primary HR (95%CI) 2.07 (1.09–3.94), P = 0.026 for fDGF and HR (95%CI) 2.41 (1.33–4.37), P = 0.004 for dDGF and the secondary composite outcome HR (95%CI) 1.58 (1.01–2.51), P = 0.047 for fDGF and HR (95%CI) 1.67 (1.05–2.66), P = 0.028 for dDGF. Patients who met criteria for both definitions had the worst prognosis, with a three‐year estimates (95%CI) of survival from the primary and secondary outcomes of 2.31 (2.02–2.59) and 2.20 (1.91–2.49) years for fDGF+/dDGF+, in comparison with the other groups (P < 0.01 for trend). fDGF provides supplementary information about graft outcomes on top of the dDGF definition in a modern series of kidney transplantation.
A 38-year-old Senegalese man with no travel history for the last 2 years presented with fever and acute kidney injury with nephrotic syndrome after an elective splenectomy. Diagnosis of ...membranoproliferative glomerulonephritis due to a chronic mixed Plasmodium infection triggered by splenectomy in a patient with hyperreactive malarial splenomegaly was confirmed.
Dermographism in a patient with fever Fustà‐Novell, Xavier; García‐Herrera, Adriana; Mascaró, José M.
International journal of dermatology,
January 2018, 2018-Jan, 2018-01-00, 20180101, Letnik:
57, Številka:
1
Journal Article
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