- Sezary syndrome manifesting as posttransplant lymphoproliferative disorder. [Journal Article]
- LRLeuk Res Rep 2018; 9:72-75
- Posttransplant lymphoproliferative disorders (PTLDs) of T-cell orgin are rare biologically heterogeneous diseases of mature lymphoid cells manifesting in immunosuppressed patients. Only a few cases o...
Posttransplant lymphoproliferative disorders (PTLDs) of T-cell orgin are rare biologically heterogeneous diseases of mature lymphoid cells manifesting in immunosuppressed patients. Only a few cases of mycosis fungoides diagnosed post allogeneic hematopoietic cell transplant (alloHSCT) have been described so far. We present a patient with myelodysplastic syndrome (MDS) post matched unrelated donor alloHSCT who was on long-term immunosuppressive therapy due to graft versus host disease. Three years after an alloHSCT, she developed generalized erythroderma and peripheral blood lymphocytosis. Both skin biopsy and peripheral blood flow cytometry revealed atypical CD4+ T-cell population consistent with diagnosis of Sezary syndrome. Chimerism studies revealed 100% donor engraftment. Therapy with extracorporeal photopheresis resulted in complete response in blood and skin.
- Normal serum protein electrophoresis and mutated IGHV genes detect very slowly evolving chronic lymphocytic leukemia patients. [Journal Article]
- CMCancer Med 2018 May 09
- More than 35 years after the Binet classification, there is still a need for simple prognostic markers in chronic lymphocytic leukemia (CLL). Here, we studied the treatment-free survival (TFS) impact...
More than 35 years after the Binet classification, there is still a need for simple prognostic markers in chronic lymphocytic leukemia (CLL). Here, we studied the treatment-free survival (TFS) impact of normal serum protein electrophoresis (SPE) at diagnosis. One hundred twelve patients with CLL were analyzed. The main prognostic factors (Binet stage; lymphocytosis; IGHV mutation status; TP53, SF3B1, NOTCH1, and BIRC3 mutations; and cytogenetic abnormalities) were studied. The frequencies of IGHV mutation status, cytogenetic abnormalities, and TP53, SF3B1, NOTCH1, and BIRC3 mutations were not significantly different between normal and abnormal SPE. Normal SPE was associated with Binet stage A, nonprogressive disease for 6 months, lymphocytosis below 30 G/L, and the absence of the IGHV3-21 gene rearrangement which is associated with poor prognosis. The TFS of patients with normal SPE was significantly longer than that of patients with abnormal SPE (log-rank test: P = 0.0015, with 51% untreated patients at 5.6 years and a perfect plateau afterward vs. a median TFS at 2.64 years for abnormal SPE with no plateau). Multivariate analysis using two different Cox models and bootstrapping showed that normal SPE was an independent good prognostic marker for either Binet stage, lymphocytosis, or IGHV mutation status. TFS was further increased when both normal SPE and mutated IGHV were present (log-rank test: P = 0.008, median not reached, plateau at 5.6 years and 66% untreated patients). A comparison with other prognostic markers suggested that normal SPE could reflect slowly advancing CLL disease. Altogether, our results show that a combination of normal SPE and mutated IGHV genes defines a subgroup of patients with CLL who evolve very slowly and who might never need treatment.
- Indolent, T-cell, large granular lymphocytic leukaemia in a dog presenting with severe neutropenia and an absence of lymphocytosis. [Journal Article]
- OVOpen Vet J 2018; 8(2):118-124
- In humans, large granular lymphocytic leukaemia (LGLL) is a low-grade, indolent lymphoproliferative disorder of large granular lymphocytes (LGL) associated with autoimmune disorders; including rheuma...
In humans, large granular lymphocytic leukaemia (LGLL) is a low-grade, indolent lymphoproliferative disorder of large granular lymphocytes (LGL) associated with autoimmune disorders; including rheumatoid arthritis and single or multiple cytopenias; particularly neutropenia. Therapy largely centres around immunosuppression which aims to resolve the immune-mediated secondary pathology, often without eradicating the neoplastic clone. The most effective agents appear to be cyclophosphamide, cyclosporine and methotrexate. This case report describes the presentation, diagnostics, therapeutic approach and outcome of a 6 year-old Golden Retriever presenting with severe neutropenia. Chlorambucil, prednisolone and cyclosporine failed to improve the neutropenia but subsequent cyclophosphamide resulted in a sustained albeit temporary improvement in neutrophil count and the ability to withdraw prophylactic antibacterials. This case closely mirrors the diagnostics and therapeutic response in human LGLL.
- Syphilis infection in an HIV patient presenting with leukemoid reaction: Case report and review of the literature. [Journal Article]
- IDInfect Dis Rep 2018 Mar 29; 10(1):7410
- Leukemoid reaction (LR) is an uncommon though dreadful sign for the treating physician, as it is related to increased mortality. In the few series that have addressed its incidence and clinical signi...
Leukemoid reaction (LR) is an uncommon though dreadful sign for the treating physician, as it is related to increased mortality. In the few series that have addressed its incidence and clinical significance, infectious causes count for about half of the cases of LR, the rest accounting for cancer, drugs or rarer causes. In the HIV setting, it represents an even rarer event, owing probably to the impaired granulocytic response of AIDS patients to bacterial agents. However no report exists as to the incidence of LR to the immune-restored HIV patients adequately treated with antiretroviral therapy (ART). Syphilis is a well known cause of mild lymphocytosis, though only one report of LR exists in the congenital setting. We hereby report a case of an HIV patient adequately treated with ART, who presented with LR with a lymphomonocytic preponderance after infection with treponema pallidum.
- Age-related clonal hematopoiesis and monoclonal B-cell lymphocytosis/chronic lymphocytic leukemia: a new association? [Editorial]
- HHaematologica 2018; 103(5):751-752
- Acteoside relieves mesangial cell injury by regulating Th22 cell chemotaxis and proliferation in IgA nephropathy. [Journal Article]
- RFRen Fail 2018; 40(1):364-370
- The existing therapies of IgA nephropathy are unsatisfying. Acteoside, the main component of Rehmannia glutinosa with anti-inflammatory and anti-immune effects, can improve urinary protein excretion ...
The existing therapies of IgA nephropathy are unsatisfying. Acteoside, the main component of Rehmannia glutinosa with anti-inflammatory and anti-immune effects, can improve urinary protein excretion and immune disorder. Th22 cell is involved in IgA nephropathy progression. This study was determined to explore the effect of acteoside on mesangial injury underlying Th22 cell disorder in IgA nephropathy. Serum Th22 cells and urine total protein of patients with IgA nephropathy were measured before and after six months treatment of Rehmannia glutinosa acteoside or valsartan. Chemotactic assay and co-culture assay were performed to investigate the effect of acteoside on Th22 cell chemotaxis and differentiation. The expression of CCL20, CCL22 and CCL27 were analyzed. To explore the effect of acteoside on mesangial cell injury induced by inflammation, IL-1, IL-6, TNF-α and TGF-β1 were tested. Results showed that the proteinuria and Th22 lymphocytosis of patients with IgA nephropathy significantly improved after combination treatment of Rehmannia glutinosa acteoside and valsartan, compared with valsartan monotherapy. In vitro study further demonstrated that acteoside inhibit Th22 cell chemotaxis by suppressing the production of Th22 cell attractive chemokines, i.e., CCL20, CCL22 and CCL27. In addition, acteoside inhibited the Th22 cell proliferation. Co-culture assay proved that acteoside could relieve the overexpression of pro-inflammatory cytokines, and prevent the synthesis of TGF-β1. TGF-β1 level in mesangial cells was positively correlated with the Th22 cell. This research demonstrated that acteoside can alleviate mesangial cell inflammatory injury by modulating Th22 lymphocytes chemotaxis and proliferation.
- Polyclonal B-cell lymphocytosis: Report of three cases. [Journal Article]
- CBCytometry B Clin Cytom 2018 Apr 27
- CONCLUSIONS: Diagnose of PPBL is important in order to avoid unnecessary diagnostic procedures and therapy. This article is protected by copyright. All rights reserved.
- Plasma cell leukemia presenting as "lymphocytosis". [Journal Article]
- BloodBlood 2018 Apr 26; 131(17):1994
- Varicella-zoster virus meningitis with hypoglycorrhachia in a young immunocompetent adult without rash: A case report and literature review. [Journal Article]
- IIDCases 2018; 12:104-106
- Overt aseptic meningitis rarely complicates varicella-zoster virus (VZV) reactivation in young and immunocompetent adults. Many of the cases of VZV meningitis are associated with an exanthem. We desc...
Overt aseptic meningitis rarely complicates varicella-zoster virus (VZV) reactivation in young and immunocompetent adults. Many of the cases of VZV meningitis are associated with an exanthem. We describe an otherwise healthy 36-year-old adult who had aseptic meningitis without skin rash, caused by reactivation of varicella-zoster virus. Cerebrospinal fluid (CSF) analysis revealed lymphocytosis, increased total protein, and low glucose. Diagnosis was made by polymerase chain reaction in CSF. The present case highlights the fact that VZV should be considered as a cause of aseptic meningitis with hypoglycorrhachia in healthy and young individuals, even in the absence of an exanthem.
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- Association of polygenic risk score with the risk of chronic lymphocytic leukemia and monoclonal B-cell lymphocytosis. [Journal Article]
- BloodBlood 2018 Apr 19
- Inherited loci have been found to be associated with risk of chronic lymphocytic leukemia (CLL). A combined polygenic risk score (PRS) of representative single nucleotide polymorphisms (SNPs) from th...
Inherited loci have been found to be associated with risk of chronic lymphocytic leukemia (CLL). A combined polygenic risk score (PRS) of representative single nucleotide polymorphisms (SNPs) from these loci may improve risk prediction over individual SNPs. Herein, we evaluated the association of a PRS with CLL risk and its precursor, monoclonal B-cell lymphocytosis (MBL). We assessed its validity and discriminative ability in an independent sample and evaluated effect modification and confounding by family history (FH) of hematological cancers. For discovery, we pooled genotype data on 41 representative SNPs from 1,499 CLLs and 2,459 controls from the InterLymph Consortium. For validation, we utilized data from 1,267 controls from Mayo Clinic and 201 CLLs, 95 MBLs, and 144 controls with FH of CLL from the GEC Consortium. We used odds ratios (ORs) to estimate disease associations with PRS and c-statistics to assess discriminatory accuracy. In InterLymph, the continuous PRS was strongly associated with CLL risk (OR=2.49, P=4.4×10-94). We replicated these findings in the GEC Consortium and Mayo controls (OR=3.02, P=7.8x10-30) and observed high discrimination (c-statistic=0.78). When jointly modeled with FH, PRS retained its significance, along with FH status. Finally, we found a highly-significant association of the continuous PRS with MBL risk (OR=2.81, P=9.8×10-16). In conclusion, our validated PRS was strongly associated with CLL risk, adding information beyond FH. The PRS provides a means of identifying those individuals at greater risk for CLL as well as those at increased risk of MBL, a condition that has potential clinical impact beyond CLL.