- Relapsing Polychondritis with Meningoencephalitis Refractory to Immunosuppressant Therapy. [Journal Article]
- CRCase Rep Neurol Med 2018; 2018:1873582
- Meningoencephalitis is a rare complication of relapsing polychondritis. We report a case of a 25-year-old male who presented with visual hallucinations and symptoms of depression and anxiety, white m...
Meningoencephalitis is a rare complication of relapsing polychondritis. We report a case of a 25-year-old male who presented with visual hallucinations and symptoms of depression and anxiety, white matter changes on MRI, and CSF lymphocytosis, along with inflammatory chondritis seen in his auricle cartilage biopsy. Eventually he was given the diagnosis of RP presenting with meningoencephalitis based on CSF analysis, brain MRI findings, negative serologies, and neurologic exam findings. The patient's clinical state did not improve despite being on IV methylprednisolone for a period of 7 days; afterwards he was switched to oral prednisone with no clinical improvement. As a result, he was given cyclophosphamide and rituximab, respectively, without benefit. He also underwent craniectomy with VP shunt due to worsening hydrocephalus and a brain biopsy was done to confirm the diagnosis. He is currently on methotrexate and steroid dependent with a goal to taper down. Even though all 19 reported cases of meningoencephalitis with RP in the literature did respond to immunosuppressive therapy, in our case, however the patient did not respond to immunosuppressive treatment and currently is in mute dementia status after three years of treatment.
- Serum 1,25(OH)2 Vitamin D and 25(OH) Vitamin D Ratio for the Diagnosis of Sarcoidosis-Related Uveitis. [Journal Article]
- OIOcul Immunol Inflamm 2018 Nov 05; :1-7
- CONCLUSIONS: The measurement of serum 25(OH)D and 1,25(OH)2D levels is a useful tool in the etiological workup of patients with unexplained uveitis, since a high 1,25(OH)2D/25(OH)D ratio is suggestive of ocular sarcoidosis.
- Accumulation of DNA damage and alteration of the DNA damage response in monoclonal B-cell lymphocytosis and chronic lymphocytic leukemia. [Journal Article]
- LLLeuk Lymphoma 2018 Oct 31; :1-10
- Accumulation of DNA damage and alteration of the DNA damage response (DDR) are critical features of genetic instability that is presumed to be implicated in the pathogenesis of monoclonal B-cell lymp...
Accumulation of DNA damage and alteration of the DNA damage response (DDR) are critical features of genetic instability that is presumed to be implicated in the pathogenesis of monoclonal B-cell lymphocytosis (MBL) and chronic lymphocytic leukemia (CLL). Here, we show increased numbers of γH2AX foci, a marker of DNA double-strand breaks (DSB), in CD19+ cells of CLL patients as compared to CD19+ cells of MBL patients and healthy individuals. Furthermore, numerous γH2AX/53BP1 foci in CLL cells suggest activation of error-prone non-homologous end-joining repair mechanisms. Signatures of DDR proteins further indicate alterations of the DDR in CLL in contrast to a largely regular activation in MBL and healthy controls. In summary, our results provide evidence for the stepwise accumulation of DNA damage in the progression of MBL towards CLL and suggest increased DNA damage, error-prone DNA repair and altered DDR signaling to be critical mechanisms of clonal evolution in MBL and CLL.
- Genomewide copy number alteration screening of circulating plasma DNA: potential for the detection of incipient tumors. [Journal Article]
- AOAnn Oncol 2018 Oct 29
- CONCLUSIONS: Genomewide profiling of cfDNA in apparently healthy individuals enables the detection of incipient hematological malignancies as well as clonal mosaicism with unknown clinical significance. CNA screening of cellular DNA of peripheral blood in elderly has established that clonal mosaicism for these chromosomal anomalies predicts a 5 to 10-fold enhanced risk of a subsequent cancer. We demonstrate that cfDNA screening detects CNAs, which are not only derived from peripheral blood, but even more from other tissues. Since the clinical relevance of clonal mosaics in other tissues remains unknown, long-term follow-up is warranted. Taken together, this study demonstrates that genomewide cfDNA analysis has potential as an unbiased screening approach for hematological malignancies and premalignant conditions.
- Leukocyte Cell Population Data for Hematology Analyzer-Based Distinction of Clonal-versus-Non-Clonal Lymphocytosis: A Real-World Testing Experience. [Journal Article]
- IJIndian J Hematol Blood Transfus 2018; 34(4):623-631
- Automated blood counts revealing lymphocytosis necessitate smear reviews. Even expert morphological evaluation may however, fail to differentiate a benign-versus-malignant etiology without further te...
Automated blood counts revealing lymphocytosis necessitate smear reviews. Even expert morphological evaluation may however, fail to differentiate a benign-versus-malignant etiology without further testing. Automated analyser-derived quantitative data on leukocyte cell populations remain undertested for distinguishing such etiologies. Instrument manufacturers claim that if successful, they may be used to generate software flags that help under-resourced laboratories better triage hemogram specimens requiring further testing. We tested the diagnostic accuracy of volume-conductivity-scatter (VCS) indices together with complete blood count (CBC) parameters in such scenarios. We compared LH780-derived (Beckman Coulter, FL, USA) CBC + VCS parameters from patients with clonal lymphoproliferations (n = 42, including 30 chronic lymphocytic leukemia cases) versus 83 controls with absolute or relative lymphocytosis (derivation cohort). Diagnostic performances of 11 logistic regression equations derived were subsequently evaluated on two specific validation cohorts (n = 130 and n = 1465). Clonal lymphocytoses showed significantly lower hemoglobin and higher leukocyte counts but similar lymphocyte percentages (LY %) vis-à-vis controls. The most significant, albeit overlapping predictor of clonality was the absolute lymphocyte count, LY# (47.8 ± 48.4 × 109/L vs. 2.9 ± 1.4 × 109/L in clonal vs. benign cases). In eleven logistic regression equations constructed using four combinatorial approaches, only the models with LY# (highest sensitivity/specificity of 99.3%/100%) and the lymphocytic VCS parameters alone (highest sensitivity/specificity of 76.2%/90.2%) performed consistently in both validation cohorts. Lymphocytic VCS parameters were moderately successful in distinguishing benign-versus-malignant lymphocytes. Other approaches of CBC-plus-VCS parameters did not sustain their initial excellent performances in the validation cohorts, highlighting a need for careful appraisal and better standardization of automated cellular analysis technologies.
- Study of Central Nervous System Tuberculosis. [Journal Article]
- JAJ Assoc Physicians India 2018; 66(1):41-44
- CONCLUSIONS: Diagnosis of CNS TB should be based on clinical features and 3 or more supportive criteria rather than CSF positivity on staining or culture. Rapid and early diagnosis by positive CSF PCR and CT/MRI findings should replace CSF AFB staining and culture in further for the diagnosis of CNS TB.
- Gastroduodenal Disorders in Patients with CVID Undergoing Immuno-globulin Therapy. [Journal Article]
- CPCurr Pharm Biotechnol 2018 Oct 10
- CONCLUSIONS: In our cohort of CVID patients, gastro-duodenal histopathological findings, including ma-lignancies, are frequent and can affect long-term prognosis. A rigorous endoscopic follow-up is needed in CVID patients irrespective of the gastrointestinal symptoms.
- Clinical Insignficance of Monoclonal T-Cell Populations and Duodenal Intraepithelial T-Cell Phenotypes in Celiac and Nonceliac Patients. [Journal Article]
- AJAm J Surg Pathol 2018 Oct 16
- Refractory celiac disease (RCD) is a rare condition, usually managed at specialized centers. However, gastroenterologists and pathologists in general practices are often the first to consider a diagn...
Refractory celiac disease (RCD) is a rare condition, usually managed at specialized centers. However, gastroenterologists and pathologists in general practices are often the first to consider a diagnosis of RCD in celiac patients with persistent symptoms. The distinction between type I and type II RCD is crucial as patients with RCD II have a shortened life expectancy. The diagnosis of RCD II requires the demonstration of abnormal intraepithelial lymphocytes and/or monoclonal T-cell populations in duodenal biopsies, typically assessed in formalin-fixed paraffin-embedded tissue. We investigated the clinical significance of T-cell receptor gene rearrangements and CD3/CD8 staining in formalin-fixed paraffin-embedded biopsies from 32 patients with RCD I (4), RCD II (3), newly diagnosed celiac disease (CD) (10), established CD patients with follow-up biopsies (10), and Helicobacter pylori-associated lymphocytosis (5). Clonal T-cell populations were present in all lymphocytosis groups but not in normal controls. No difference in the frequency of clonal populations or persistence of identical clones was found between RCD I and II patients. The degree of villous blunting did not correlate with clonal status in any group. No difference in the number of CD3/CD8-positive intraepithelial lymphocytes per 100 enterocytes was found between groups. We suggest that clonal evaluation of T cells should not be employed routinely in the evaluation of CD patients with persistent symptoms until common causes of "apparent refractoriness" have been excluded. In addition, lymphocyte phenotyping and T-cell clonal analysis appear to be insufficient as stand-alone tests to reliably distinguish RCD I and II.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0/.
- Quantification and phenotypic characterization of peripheral blood Vδ1 + T cells in chronic lymphocytic leukemia and monoclonal B cell lymphocytosis. [Journal Article]
- CBCytometry B Clin Cytom 2018 Oct 17
- CONCLUSIONS: These experiment findings point to an expansion of CD27- Vδ1+ T cells with a cytotoxic profile, from controls to advanced stages of the disease, which points to a role of Vδ1+ T cells in the host's anti-tumor responses against clonal B-cells in MBL and CLL. © 2018 Clinical Cytometry Society.
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- Novel association of Streptococcus gallolyticus subspecies pasteurianus and hepatocelluar carcinoma: opening new frontiers. [Journal Article]
- SJScand J Gastroenterol 2018 Oct 18; :1-4
- CONCLUSIONS: This study provides a novel insight into the association of Streptococcus gallolyticus subspecies pasteurianus with HCC. The isolation of the organism from blood and ascitic fluid should prompt the clinicians to search for evidence of HCC actively.