Presence Of Atypical Lymphocytes Research Articles

Lymphomatosis cerebri caused by adult T cell leukemia/lymphoma: a differential diagnosis for depression: a case report

BackgroundPrimary central nervous system lymphoma is rare, and primary central nervous system T cell lymphoma is relatively uncommon, contributing to < 5% of all cases. Lymphomatosis cerebri, a rare subtype of primary central nervous system lymphoma, is characterized by extensive white-matter lesions on magnetic resonance imaging and nonspecific symptoms, such as cognitive decline and depression. Reports of lymphomatosis cerebri in adult T cell leukemia/lymphoma are limited.Case presentationA 49-year-old Japanese man gradually developed insomnia, anorexia, and weight loss over a 2-month period following work-related promotion. Initially diagnosed with depression, his condition rapidly deteriorated with cognitive decline and motor dysfunction. Despite various treatments, his symptoms persisted within a month. Upon admission, the presence of neurological abnormalities suggestive of a central nervous system disorder raised suspicion of a cerebral lesion. Diagnostic tests revealed extensive brain lesions on imaging and the presence of atypical lymphocytes (flower cells) in the cerebrospinal fluid. The patient was diagnosed with lymphomatosis cerebri due to adult T cell leukemia/lymphoma, a rare presentation in the literature. Due to irreversible brainstem damage and poor neurological prognosis, aggressive treatment was not initiated, and the patient died, with an autopsy confirming the diagnosis.ConclusionLymphomatosis cerebri with adult T cell leukemia/lymphoma is very rare. It is crucial to promptly consider lymphomatosis cerebri as a differential diagnosis, particularly in cases of rapid cognitive decline and poor treatment response. Recognition of lymphomatosis cerebri as an important differential diagnosis for cognitive decline, and depression is necessary for timely intervention and management. Further research is required to better understand this unique and rare presentation in adult T cell leukemia/lymphoma.

Histology-based classifier to distinguish early mycosis fungoides from atopic dermatitis.

Histopathological differentiation of early mycosis fungoides (MF) from benign chronic inflammatory dermatoses remains difficult and often impossible, despite the inclusion of all available diagnostic parameters. To identify the most impactful histological criteria for a predictive diagnostic model to discriminate MF from atopic dermatitis (AD). In this multicentre study, two cohorts of patients with either unequivocal AD or MF were evaluated by two independent dermatopathologists. Based on 32 histological attributes, a hypothesis-free prediction model was developed and validated on an independent patient's cohort. A reduced set of two histological features (presence of atypical lymphocytes in either epidermis or dermis) was trained. In an independent validation cohort, this model showed high predictive power (95% sensitivity and 100% specificity) to differentiate MF from AD and robustness against inter-individual investigator differences. The study investigated a limited number of cases and the classifier is based on subjectively evaluated histological criteria. Aiming at distinguishing early MF from AD, the proposed binary classifier performed well in an independent cohort and across observers. Combining this histological classifier with immunohistochemical and/or molecular techniques (such as clonality analysis or molecular classifiers) could further promote differentiation of early MF and AD.

Keratic precipitates morphology in vitreoretinal lymphoma evaluated with in-vivo confocal microscopy

A 51-year-old female patient presented to our department with a one-year history of bilateral blurred vision with floaters. Ocular examination showed bilateral vitritis with a moderate haze which was more accentuated and prominent at the periphery in the right eye (OD) (figure 1 A, asterisks). Spectral-domain optical coherence tomography (SD-OCT) showed numerous pigment epithelial detachment (figure B, white arrows), with a lumpy-bumpy choroid. The diagnosis of vitreoretinal lymphoma (VRL) was suspected and confirmed histologically by the presence of atypical lymphocytes in the vitreous samples and an elevated Interleukin (IL)-10 to IL-6 ratio. In addition to those posterior segment manifestations, slit-lamp examination of the OD showed central stellate granulomatous keratic precipitates (KPs) (figure C). On in-vivo confocal microscopy (IVCM), those infiltrating KPs appeared as a hyperreflective deposit at the level of the corneal endothelium with a dendritic shape, and were interconnected by multiple thin and fibrillar pseudopodia in a comet-like appearance (figure D, yellow arrows), which is a highly suggestive pattern of KPs of VRL associated anterior uveitis when the latter is unusually granulomatous.

Peripheral blood cell anomalies in COVID-19 patients in the United Arab Emirates: A single-centered study.

In this study, we aimed at exploring the morphologic and quantitative abnormalities in the peripheral blood counts of coronavirus disease 2019 (COVID-19) patients. A cohort of 131 COVID-19 patients was recruited at University Hospital Sharjah (UHS), UAE. Their peripheral blood smears were examined for morphological evaluation. Also, their clinical laboratory investigations and radiological findings were retrieved from the medical records. Our cohort consisted of 63 males and 68 females with an age of 63.6 ± 18.6 years. The presence of atypical lymphocytes was observed in around 80% of the recruited COVID-19 patients. Further, monocytes with toxic cytoplasmic vacuoles were identified in 55% of the cases. Neutrophil-associated changes, including pseudo-Pelger-Huët, bands, and long nuclear endoplasm, were reported in around 25-35% of the patients. RBCs associated changes such as microcytic and hypochromic RBCs, as well as targetoid, dacrocytes, ovalocytes, echinocytes/burr cells, and schistocytes, were described. According to disease severity, RBCs chromicity was found to be significantly different between stable and critical patients. COVID-19 patients with CO-RADS 5 showed a similar change in RBCs as well as a decrease in the neutrophils with hypogranular cytoplasm. Peripheral blood smear assessment in COVID-19 patients could provide information about the disease state and pulmonary involvement.

Hematological changes in human lymphotropic-T virus type 1 carriers.

The human T-lymphotropic virus type 1 (HTLV-1), isolated in 1980, causes T-cell leukemia/lymphoma in adulthood, a type of lymphoproliferative disease, and chronic HTLV-1-associated myelopathy, a disease that causes paralysis of the lower limbs, which occur in about 5% of cases in this viral infection. This study aimed to establish the hematological profile of patients with HTLV-1 infection in Belém do Pará, describing the hematological parameters under study, estimating the frequency of lymphocytic atypical, and associating the hematological profile with diseases and symptoms. Hematologic data from 202 individuals were analyzed, including 87 HTLV-1 infected individuals and 115 non-HTLV-1 infected individuals as a control group, composed, at a great part, of relatives of the infected. The seroprevalence of HTLV-1 infection was observed in 71.3% of female individuals, with predominance in the group older than 50 years (44.8%). The analysis of hematological parameters showed a significant difference in the counts of the segmented cells (p = 0.0303) and eosinophils (p = 0.0092) in HTLV-1 carriers. Lymphocytic atypical was a finding present only in HTLV-1 carriers (p = 0.0001). There was no high frequency in the leukocyte counts of those infected by HTLV-1 not among them concerning a significant increase or decrease. It is concluded that HTLV-1 infection is prominent in women over 50 years old. The hematological profile of those infected shows a reduction of segmented cells, an increase of eosinophils, and the presence of atypical lymphocytes. The hematological profile of the HTLV-1 carrier should always be evaluated to identify early some diseases associated with the infection.

Challenges of Primary Care Medicine in a Tertiary Care Setting-The Case of Primary CMV Infection Compared to Primary EBV Infection: A Retrospective Cohort Study.

BackgroundIn the immunocompetent adult primary cytomegalovirus (CMV) infection may present as prolonged febrile illness or may resemble infectious mononucleosis. Hence, establishing a diagnosis of primary CMV infection may be challenging, in particular in the hospital setting.MethodsWe performed a retrospective analysis of all immunocompetent patients treated at a tertiary care center in Switzerland over a 5-year period in whom a diagnosis of primary CMV infection was established. We assessed their demographic, clinical, and laboratory characteristics and compared them to patients with a diagnosis of primary Epstein-Barr virus (EBV) infection during the same period.ResultsWe identified 16 and 125 patients with primary CMV and EBV infection, respectively (rates of 3.1 and 23.8 cases/year, respectively). Patients in the CMV group were older (median 34 vs. 22 years), had a longer illness duration before presentation (median 14 vs. 7 days) and more frequently systemic symptoms compared to patients in the EBV group. Increased lymphocyte count and presence of atypical lymphocytes were observed in both groups, yet less frequently and less pronounced in the CMV group. The overall number of performed tests (including laboratory and radiology tests) was significantly higher in the CMV group (median 11.5 vs. 3.0) before arriving at the final diagnosis. Antibiotic treatment was more frequently prescribed in patients with primary EBV infections (40 vs. 25%).ConclusionsGiven its low incidence and non-specific symptoms, establishing a diagnosis of primary CMV infection can be challenging. Knowledge about clinical features of primary CMV infection in the immunocompetent host might help to adopt a stepwise approach to diagnosis avoiding over-testing.

Evaluation of sensitivity and specificity of high fluorescence lymphocyte count percentage of sysmex XN analyzer in diagnosis of dengue

BACKGROUND: The Sysmex XN-series hematology analyzers provide newer parameters including high fluorescence lymphocyte cell percentage (HFLC%) which correlates with the presence of atypical lymphocytes in peripheral blood. We aimed to analyze the sensitivity and specificity of HFLC% as a diagnostic tool and its association with serological status in diagnosed dengue patients and thereby establish a cutoff of HFLC% based on serology. Besides, we also wish to correlate HFLC% with thrombocytopenia in these patients.MATERIALS AND METHODS: A total of 1500 serum samples were subjected to serological evaluation for dengue. After excluding hematological malignancies and autoimmune disorders, the same day complete blood count parameters including HFLC% and platelet counts were collected retrospectively for 292 serologically positive dengue cases and 76 seronegative controls.RESULTS: Our result shows that in nonstructural 1 antigen-positive cases, a cutoff of >5.2% HFLC can have a sensitivity of 79.5% and specificity of 98.6%. We found a different cut off of HFLC% >3.2% (sensitivity 83.4%, specificity 98.6%) for the cases with only immunoglobulin M positivity and a cut off of HFLC% >2.6% (sensitivity 86.1%, specificity 96%) in the dual positive cases (immunoglobulin M with nonstructural 1 antigen). Besides, high HFLC% also shows a strong correlation with platelet count with a Spearman correlation coefficient of −0.6.CONCLUSIONS: The result of our study shows that a specific cutoff of HFLC% can not only help us to suspect dengue fever but also predict the risk of thrombocytopenia in already diagnosed dengue patients. The sensitivity and specificity of HFLC% varied with the serological status of the patients which depend on the days of fever on presentation.

Atypical lymphocytes in the peripheral blood of COVID-19 patients: A prognostic factor for the clinical course of COVID-19.

Clinical observations have shown that there is a relationship between coronavirus disease 2019 (COVID-19) and atypical lymphocytes in the peripheral blood; however, knowledge about the time course of the changes in atypical lymphocytes and the association with the clinical course of COVID-19 is limited. Our purposes were to investigate the dynamics of atypical lymphocytes in COVID-19 patients and to estimate their clinical significance for diagnosis and monitoring disease course. We retrospectively identified 98 inpatients in a general ward at Kashiwa Municipal Hospital from May 1st, 2020, to October 31st, 2020. We extracted data on patient demographics, symptoms, comorbidities, blood test results, radiographic findings, treatment after admission and clinical course. We compared clinical findings between patients with and without atypical lymphocytes, investigated the behavior of atypical lymphocytes throughout the clinical course of COVID-19, and determined the relationships among the development of pneumonia, the use of supplemental oxygen and the presence of atypical lymphocytes. Patients with atypical lymphocytes had a significantly higher prevalence of pneumonia (80.4% vs. 42.6%, p < 0.0001) and the use of supplemental oxygen (25.5% vs. 4.3%, p = 0.0042). The median time to the appearance of atypical lymphocytes after disease onset was eight days, and atypical lymphocytes were observed in 16/98 (16.3%) patients at the first visit. Atypical lymphocytes appeared after the confirmation of lung infiltrates in 31/41 (75.6%) patients. Of the 13 oxygen-treated patients with atypical lymphocytes, approximately two-thirds had a stable or improved clinical course after the appearance of atypical lymphocytes. Atypical lymphocytes frequently appeared in the peripheral blood of COVID-19 patients one week after disease onset. Patients with atypical lymphocytes were more likely to have pneumonia and to need supplemental oxygen; however, two-thirds of them showed clinical improvement after the appearance of atypical lymphocytes.

Hematological and Morphological Changes in the Peripheral Blood Smear of Patients with COVID-19

Background: The novel coronavirus is responsible for a widespread pandemic, causing multiple clinical manifestations. Peripheral blood films have shown changes in white blood cell (WBC) morphology and counts, as well as platelet abnormalities. Atypical and reactive lymphocytes may also be observed in the blood film, which suggests mild leukoerythroblastosis. Objectives: The present study aimed to evaluate the hematological and morphological changes in the peripheral blood smear of patients with COVID-19. Methods: This study was conducted on 175 patients diagnosed with COVID‐19 infection. Blood samples were collected from the patients and examined for the estimation of blood parameters, differential cell count, and presence of atypical lymphocytes. Results: The mean age of the patients was 53.73 years, and males constituted 52.6% of the patients. The majority had normal hemoglobin levels. WBC count was normal in 60.6% of the patients, while 74.3% had normal platelet count, and thrombocytopenia was detected in 23.4%. In addition, differential WBC count indicated that 56.6% of the patients had normal neutrophils, while lymphopenia was detected in 28 and 77.7% had a low eosinophil count. The peripheral blood smears showed atypical lymphocytes with deeply basophilic cytoplasm in 21.14% of the patients. Conclusions: Blood film examination may show multiple abnormal findings in patients with COVID-19 infection. Neutrophilia and lymphopenia with the presence of atypical lymphocytes are frequently observed as well. Low eosinophil count is also highly common. Although platelets showed changes, they were normal or reduced in most cases.

Comprehensive evaluation of bronchoalveolar lavage from patients with severe COVID-19 and correlation with clinical outcomes

Information on bronchoalveolar lavage (BAL) in patients with COVID-19 is limited, and clinical correlation has not been reported. This study investigated the key features of BAL fluids from COVID-19 patients and assessed their clinical significance. A total of 320 BAL samples from 83 COVID-19 patients and 70 non-COVID-19 patients (27 patients with other respiratory viral infections) were evaluated, including cell count/differential, morphology, flow cytometric immunophenotyping, and immunohistochemistry. The findings were correlated with clinical outcomes. Compared to non-COVID-19 patients, BAL from COVID-19 patients was characterized by significant lymphocytosis (p < 0.001), in contrast to peripheral blood lymphopenia commonly observed in COVID-19 patients and the presence of atypical lymphocytes with plasmacytoid/plasmablastic features (p < 0.001). Flow cytometry and immunohistochemistry demonstrated that BAL lymphocytes, including plasmacytoid and plasmablastic cells, were composed predominantly of T cells with a mixture of CD4+ and CD8+ cells. Both populations had increased expression of T-cell activation markers, suggesting important roles of helper and cytotoxic T-cells in the immune response to SARS-CoV-2 infection in the lung. More importantly, BAL lymphocytosis was significantly associated with longer hospital stay (p < 0.05) and longer requirement for mechanical ventilation (p < 0.05), whereas the median atypical (activated) lymphocyte count was associated with shorter hospital stay (p < 0.05), shorter time on mechanical ventilation (p < 0.05) and improved survival. Our results indicate that BAL cellular analysis and morphologic findings provide additional important information for diagnostic and prognostic work-up, and potential new therapeutic strategies for patients with severe COVID-19.

Rare Case Report of Adult T-Cell Leukemia / Lymphoma (ATLL), in the City of Macau- Rio Grande Do Norte; Brazil

Introduction:Adult T-cell leukemia / lymphoma (ATLL) is a rare type of malignant haemopathy with the Human T-Cell Lymphotropic Virus type I (HTLV-1) as its etiologic agent. The clinical picture includes skin lesions, hypercalcemia, elevated lactate dehydrogenase (LDH), adenomegaly, myelopathy and leukocytosis with atypical TCD4 lymphocytes.Objective:To report a case of a patient from the city of Macau-RN, with ATLL, presenting skin lesions, weight loss (&amp;gt; 13%), leukocytosis and supraclavicular lymph node enlargement. Case Description: Male patient, bricklayer, 67-year-old, hypertensive, was admitted to a hospital in Natal-RN, with severe itching, diffuse exanthematic skin lesions for 13 days, adynamia, associated with progressive, weight loss (P: 73Kg, 63Kg -13.6%), hyporexia, feverish (37.8 ° C), insidious and progressive drowsiness. Physical examination showed palpable lymphadenomegaly in the right supraclavicular chain, 1 cm, mobile and painless. Painful abdomen on deep palpation in the right hypochondrium and left iliac fossa, moderate hepatosplenomegaly. The blood count showed leukocytosis with the presence of atypical lymphocytes with convoluted nucleus (Flow-Cell), Creatinine (6.81), urea 349, hyperuricemia (14.9 mg / dL), hypercalcemia (12.8), LDH (723), Alkaline phosphatase (1388) GGT (183). Abdominal, kidney and urinary tract ultrasound revealed acute pancreatitis with signs of saponification (steatonecrosis of peripancreatic fat) and bilateral acute parenchymal nephropathy, respectively. The transthoracic echocardiogram identified aortic and mitral valve sclerosis. Immunophenotyping by flow cytometry found lymphoproliferative disease of mature TCD4 + cells, and the diagnosis of ATLL was confirmed by the serological reagent for HTLV-1. From then on, adequate treatment for ATLL was started with reversion of the clinical period, currently being followed up on an outpatient basis.Conclusions:The multiprofessional performance with accurate clinical investigation associated and specific laboratory exams such as immunophenotyping by flow cytometry and serological tests are essential for the early diagnosis and treatment of ATLL, a rare entity in the state of Rio Grande do Norte., Brazil and that high mortality when not diagnosed and treated early. Disclosures No relevant conflicts of interest to declare.

MCP-1 LEVELS AND ATYPICAL LYMPHOCYTES IN EARLY FEVER OF DENGUE VIRUS INFECTION WITH NON-STRUCTURAL PROTEIN 1 (NS-1) ANTIGEN TEST IN dr DARSONO HOSPITAL, PACITAN

Dengue infection caused by DENV and transmitted by mosquitoes Aedes aegypti and Aedes albopictus is a major health problem in the world, including Indonesia. Clinical manifestations of dengue infection are very widely, from asymptomatic until dengue shock syndrome (DSS). DENV will attack macrophages and dendritic cells (DC) and replicate them. Monocytes are macrophages in the blood (±10% leukocytes). Macrophages produce cytokines and chemokines such as monocyte chemotactic protein-1 (MCP-1)/CCL2. The monocytes that are infected with DENV will express MCP-1, which will increase the permeability of vascular endothelial cells so that they have a risk of developing DHF/DSS. Macrophages and DC secrete NS1 proteins, which are the co-factors that are needed for viral replication and can be detected in the early phase of fever. The increased MCP-1 levels in dengue infection followed by an increase in the number of atypical lymphocytes indicate the arrival of macrophages and monocytes to the site of inflammation which triggers proliferation rather than lymphocytes. This is an observational analytical study with a cross-sectional design to determine the MCP-1 level in dengue infection patients with 1st until the 4th day of fever and the presence of atypical lymphocytes. Dengue infection was determined by rapid tests NS1 positive or negative and MCP-1 levels were measured using by ELISA sandwich method.MCP-1 level of sixty patients dengue infection NS-1 rapid positive or negative with 2nd until 4rt fever were significantly higher than healthy subjects (420.263±158,496vs29, 475±23.443;p=0.000), but there was no significant difference in subjects with DF, DHF or DSS (436,47±225,59 vs422,77±170,55vs 448,50±117,39; p =0.844). Atypicallymphosite differs significantly in healthy subjects than subjects infected with DENV an average of 2% (p= 0,000). In conclusion, this shows the arrival of macrophages and monocytes to the site of inflammation, which triggers the proliferation of lymphocytes.

Acute cytomegalovirus infection in an immunocompetent patient with ulcerative colitis: A case report

Cytomegalovirus (CMV) is a ubiquitous member of the Herpesviridae family that can present with a variety of clinical manifestations, including encephalitis, retinitis, interstitial pneumonia and colitis. These serious symptoms are generally observed as opportunistic infections in immunocompromised hosts, including patients with acquired immunodeficiency syndrome and those receiving steroids and/or immunosuppressants. Symptomatic CMV infections in patients with ulcerative colitis are found in patients treated with steroids and/or immunosuppressants but rarely affect those who are not taking these agents. The present study reported the case of a young patient without concurrent use of immunosuppressive agents for the treatment of ulcerative colitis. The patient presented with acute mononucleosis and colitis caused by primary CMV infection. This was characterized by the presence of atypical lymphocytes and hepatosplenomegaly, elevation of transaminase levels, serology-positive anti-CMV IgM, and CMV antigenemia. Additionally, CMV-positive cells were histologically detected in colonic biopsy specimens. The patient's symptoms and clinical parameters improved following initiation of intravenous ganciclovir. It was concluded that even if patients with ulcerative colitis are not treated with steroids and/or immunosuppressants, significant attention should be paid to acute CMV infections in the context of severe or persistent colonic inflammation.

Maximising yield of peripheral blood flow cytometry for chronic lymphoproliferative disorders.

Flow cytometry is used in the diagnosis of haematological diseases including chronic lymphoproliferative disorders. This audit aims to ascertain the real-world indications for peripheral blood (PB) flow cytometry and which of these are associated with higher diagnostic yields. All PB flow cytometry requests for chronic lymphoproliferative disorders from 1 January 2014 to 31 December 2014 were identified using the laboratory information system. Data including patient demographics, specialty of requestor, lymphocyte count and blood film report (if available), indications for tests and subsequent diagnosis were collected. A total of 185 requests with median patient age of 60years were analysed. The main requestor was the Haematology Unit (n=109; 58.9%) although the diagnostic yield of their requests was not significantly better than other units combined (16.5% vs 13.2%, P=.49). Factors that significantly improved the diagnostic yield of testing were older age, the presence of atypical lymphocytes on the blood film and lymphocytosis (P<.01). Constitutional symptoms and cytopenias were not found to influence the diagnostic yield. PB flow cytometry is a useful tool when used in the appropriate clinical setting. Rationalisation of testing is important to reduce the futility of testing and unnecessary health costs.

A retrospective study of DRESS - drug reaction with eosinophilia and systemic symptoms.

DRESS (drug reaction with eosinophilia and systemic syndrome) is qualified as hypersensitivity after drug reaction. This syndrome may occur due to any medication intake. There are three main groups of symptoms defining DRESS: skin lesions, hematological abnormalities and internal organ involvement. A retrospective study was performed on a group of 261 patients with drug reactions hospitalized in the Clinic of Dermatology from 2004 until 2017. There were ten cases of DRESS of 261 hypersensitivity drug reactions observed in the Clinic. The drug which most frequently caused DRESS in the studied group was carbamazepine - six patients (60%). Lamotrigine was the cause of DRESS in two cases, oxycarbamazepine in one patient and dexketoprofen in one patient. The skin lesion was present in 100% patients. Mainly it was coalescing hemorrhagic rash accompanied by face edema. Eosinophilia was noticed in 80% of patients and the presence of atypical lymphocytes - in 40%. The main infiltrate organ was liver in 8 cases. DRESS diagnosis should be taken into consideration especially in patients treated eith antiepileptic drugs. Early diagnosis and drug discontinuation can contribute to preventing serious complications of DRESS.

Infectious mononucleosis due to epstein-barr virus infection in children: A profile from eastern India

Objective: The objective of this study is to delineate the clinical and laboratory profile of infectious mononucleosis due to Epstein-Barr virus (EBV) infection in children admitted to tertiary care teaching hospitals. Materials and Methods: Retrospective observational multicentric analysis of clinical and laboratory features of children between 1 month to 12 years with a diagnosis of infectious mononucleosis due to EBV infection confirmed by positive serology over a 12-month period after seeking approval from the Institutional Ethics Committee. Results: Out of 66 children screened, 53 were included in final analysis. The majority were aged between 5 and 8 years with male: female ratio of 1.2:1. Most presentations were during the monsoon months. The common clinical features were fever (100%), splenomegaly (86.7%), and cervical lymphadenopathy (73.5%) in contrast to the classical triad of fever, sore throat, and generalized lymphadenopathy described in the literature. There were no age differences in clinical findings except for generalized and cervical lymphadenopathy and hepatomegaly which were commoner in 9–12 years age band. Although the incidence of common findings matched with previously published studies, there were some notable differences. While frequencies of upper eyelid edema, epitrochlear lymphadenopathy, and splenomegaly were more, those of rash and sore throat were less. Lymphocytosis and presence of atypical lymphocytes were relatively less common in our series. All children recovered. Conclusions: This multicentric study on profiling childhood infectious mononucleosis, possibly first of its kind from Eastern India, has documented clinical and laboratory features associated with this condition. These data can serve as a reference for future studies.

Splenic Marginal Zone Lymphoma: A Case Report with Review of Literature Running Title: A Rare Case Report On Splenic Marginal Zone Lymphoma

Splenic marginal zone lymphoma(SMZL) is a rare B-cell neoplasm comprising less than 2% of lymphoid neoplasms ,composed of small lymphocytes which surround and replace the splenic white pulp ,germinal centres,follicle mantle and merge with marginal zone.We report a case of SMZL in a 40 year old male patient presenting with complaints of low grade fever and left sided abdominal discomfort for 6 months and was noted to have massive splenomegaly on physical examination. Laboratory evaluation revealed leucocytosis and presence of atypical lymphocytes which were larger than mature lymphocytes having moderately basophilic cytoplasm with cytoplasmic processes at the poles of the cell with round to oval nucleus with clumped chromatin and indistinct nucleoli.Immunophenotypic analysis revealed a B-cell population with CD -20 positivity.

Drug reaction with eosinophilia and systemic symptoms (DRESS): A histopathology based analysis.

The data on the histology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) is limited. To study the histopathology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) and to identify any features with diagnostic or prognostic significance. All patients admitted to the dermatology ward of government medical college, Kozhikode from January 1, 2014 to December 31, 2014 with probable or definite DRESS as per the RegiSCAR scoring system and who were willing to undergo skin biopsy were included in this prospective study. The study population comprised of nine patients. The consistent histological finding documented was the predominantly lymphocytic dermal inflammatory infiltrate. Four of the five patients whose histology revealed focal interface dermatitis and keratinocyte vacuolation with or without apoptotic keratinocytes, had elevated liver transaminases. Tissue eosinophilia was associated with disease flares. The presence of atypical lymphocytes in peripheral smear and histological evidence of dense dermal inflammatory infiltrate showed an association with hepatic involvement. The main limitations of our study were the small sample size and our inability to carry out a detailed immunohistochemistry work-up. In the appropriate setting, varying combinations of epidermal hyperplasia, spongiosis, parakeratosis and individually necrotic keratinocytes in the background of lymphocyte predominant dermal infiltrate (with some atypia) favor a diagnosis of drug reaction with eosinophilia and systemic symptoms. Female sex, the presence of atypical lymphocytes in peripheral smear, dense dermal inflammatory infiltrate, tissue eosinophilia and interface dermatitis with or without keratinocyte necrosis was associated with a poor prognosis.

Profound Hypereosinophilia Secondary to Sezary Syndrome

Background: Sezary syndrome is a subtype of cutaneous non-Hodgkin's lymphoma that is characterized by erythroderma, the presence of atypical lymphocytes in the blood, and lymphadenopathy. The clonal cell in Sezary syndrome is thought to be derived from mature Th-2 cells that are able to express Il-4, Il-5, and Il-13 which can stimulate the replication of eosinophils. It is has been shown that higher levels of eosinophilia correlate with a worse prognosis. One explanation is that the degree of eosinophilia represents the level of imbalance between Th-1 and Th-2 cells. Here we report the first case of end organ damage caused by hypereosinophilia secondary to the Sezary syndrome. In rare cases Sezary syndrome can cause hypereosinophilia but has never been reported to cause hypereosionphilia that results in end-organ damage. To our knowledge this is the first case that reports end-organ damage resultant from secondary hypereosinophilia from the Sezary syndrome.Case presentation: A 75-year-old man was admitted for severe rash and pruritis, massive lymphadenopathy, and extremely high eosinophil count. He had recurrent skin rash with pruritis for five years with previous skin biopsies without evidence of malignancy. He had never had hypereosinophilia until he presented to our institution. Peripheral smear showed marked eosinophilia as well as the presence of Sezary cells. A skin biopsy and lymph node biopsy revealed cutaneous T cell lymphoma. Bone marrow biopsy showed atypical large T-cells comprising 29% of the total population with loss of CD5 and CD7 and 22% eosinophils. The patient ultimately sustained multiorgan failure and expired from respiratory failure. Tissue eosinophilia was later demonstrated on autopsy.Conclusion: Our report indicates that the Sezary syndrome can cause extreme hypereosinophilia that can result in end-organ damage. DisclosuresNo relevant conflicts of interest to declare.

Drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome: clinical features of 27 patients.

Drug reaction with eosinophilia and systemic symptoms (DRESS) [also called drug-induced hypersensitivity syndrome (DIHS)] includes severe reactions to drugs that need to be promptly recognized by physicians. To explore heterogeneity in the clinical presentation of DRESS/DIHS at a large academic hospital in Latin America, using the criteria defined by the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system. A retrospective medical record review of 60 patients with diagnostic suspicion of DRESS/DIHS admitted to our hospital between July 2008 and April 2012 was performed, including demographic data, clinical features, laboratory findings and treatment. Of the 60 patients, 27 fulfilled the criteria for DRESS/DIHS. Maculopapular exanthema (85.1%), fever (96.2%) and hepatic involvement (85.1%) were the most common features. Anticonvulsants were the most common causal drugs (77.7%); Phenytoin was the most common individual drug (44.4%), followed by carbamazepine (29.6%). All patients were treated initially with prednisone 1 mg/kg/day. Mortality rate was 4%. The major findings of this study (to our knowledge the largest collection of data on DRESS/DIHS in Latin America) include a positive statistical association between presence of atypical lymphocytes and higher levels of alanine aminotransferase (P < 0.001) and reinforce the importance of anticonvulsants in the pathogenesis of this severe reaction.