Clinicopathologic study of Kikuchi's disease in children in a tertiary hospital in South India

Background: The aim of this study was to evaluate the clinical and laboratory characteristics, treatment modalities and outcome of children with Kikuchi's disease.Methods: A retrospective cross-sectional study was conducted among all children, histopathologically diagnosed with KFD.' Clinical, laboratory data and treatment outcomes were analysed.Results: During the study period, 53 children histopathologically confirmed as KFD were enrolled in the study. There were 36 males and 17 females. The lymph node involvements were mostly cervical with bilateral predisposition (63.5%), firm (88%), matted (30.8%) and tenderness (38.5%). Fever, headache, vomiting, chills, myalgia and rash were other common presentations other than cervical lymphadenopathy. The associated laboratory findings include anemia (71.2%), leukopenia especially lymphopenia (31.4%), monocytosis (21.6%), thrombocytopenia (16.3%), elevated CRP (53.1%), ESR (83.7%), LDH (100%) and elevated liver enzymes. Most of the children were managed conservatively (49.1%). Corticosteroids were administered for (22.6 %) of patients. Recurrence occurred in 4 children (7.5 %) and 13 children (24.5%) had other associated diseases.Conclusions: KFD should be suspected in well children with febrile cervical lymphadenopathy, especially with leukopenia, monocytosis, and elevated CRP, ESR, LDH, Liver enzymes. KFD in children can have rarely atypical presentations and coexist with other diseases.

Acute monoblastic leukemia with abnormal eosinophils and inversion (16): A rare entity.

Acute myeloid leukemia (AML) is a malignant hematopoietic stem cell disorder which is sub‑classified based on bone marrow morphology and the presence of specific genetic abnormalities. One such cytogenetic abnormality is the pericentric inversion (inv) of chromosome 16 which is typically seen in AML M4 with eosinophilia and is associated with a favorable prognosis. We report the inv (16) in a young woman with AML M5 and abnormal eosinophils. This is a rare entity with only about 20 cases being reported till date.

Extracavitary primary effusion lymphoma: A case report from India.

We present a case of extracavitary primary effusion lymphoma presenting, as jejunal polyps in a 38-year-old man. This is the first report of this entity from India. Although rare in our country, the diagnosis should be suspected in cases of CD20 negative large cell lymphoma with plasmablastic or immunoblastic differentiation in seropositive patients. Immunostaining for latency-associated nuclear antigen-1 and in situ hybridization for Epstein-Barr virus-associated RNA will confirm the diagnosis.

Case of pulmonary pneumocytoma: A probable cytological diagnosis with histopathological confirmation.

Pneumocytoma is a rare benign tumor of the lung that usually presents as a solitary pulmonary nodule. It is believed to arise from the primitive undifferentiated respiratory epithelium. We report a case of pulmonary pneumocytoma that was suspected on needle aspiration smears and confi rmed histologically. This case describes the cytological features of pneumocytoma that are rarely described in textbooks.

Glomerulopathy in a patient with sarcocystis infestation.

To date, sarcocystis has been considered an asymptomatic infection in humans. Even though cases with glomerulonephritis have been reported in animals with sarcocystis, there have been no reports of a similar occurrence in humans. We report a case of acute proliferative glomerulonephritis and leukocytoclastic vasculitis in a patient with sarcocystis infestation.

Lennert's lymphoma: Clinicopathological profile of five cases.

Background and Aim: Lennert’s lymphoma is a rare variant of peripheral T-cell lymphoma (PTCL) not otherwise specifi ed (NOS) rich in epithelioid histiocytes. This study aims to analyze the clinical, morphologic, and immunophenotypic profi le of cases of Lennert’s lymphoma from our country and determines the utility of T-cell receptor (TCR) gene rearrangement in the diagnosis. Materials and Methods: All cases diagnosed as Lennert’s lymphoma during the period of January 2001 to August 2011 were included in this study. Hematoxylin and eosin (H and E) stained slides and immunohistochemistry results were analyzed and TCR gene rearrangement was performed. Results: There were fi ve cases of Lennert’s lymphoma diagnosed in our institution during this period, which included two males and three females. All cases showed effacement of lymph node architecture by diffuse infi ltration of small lymphoid T cells [CD3+, CD4+, CD8+, T-cell intracellular antigen 1 (TIA-1+), Granzyme B−] and clusters of epithelioid histiocytes throughout the lymph node and scattered large transformed cells (CD20−, CD30+, CD15−/+). TCR rearrangement was done in three cases by polymerase chain reaction (PCR) and showed the presence of a clonal T-cell population. Conclusions: Lennert’s lymphoma constituted 0.11% of all non-Hodgkin lymphomas (NHLs) in our institution. Differentiation from classical Hodgkin’s lymphoma is sometimes diffi cult by morphology and immunohistochemistry alone and TCR gene rearrangement was extremely useful in diagnosis.

Gastrointestinal lymphomas: Pattern of distribution and histological subtypes: 10 years experience in a tertiary centre in South India.

Background and Aim: Gastrointestinal tract (GIT) is one of the major sites of extra-nodal lymphomas constituting 10-15% of all non-Hodgkin's lymphoma cases and about 30-40% of extra-nodal lymphomas. Considerable variation exists in the literature with respect to incidence of the various histological subtypes and sites of involvement. This study was undertaken to ascertain the anatomic distribution, histological subtypes and sites of all GIT lymphomas presenting to a tertiary referral hospital in southern India. Materials and Methods: The histological material of 361 patients over a period of 10 years (2001-2010), with histopathological diagnosis of lymphoma involving the GIT (both primary and secondary), was analyzed retrospectively. All lymphomas were reclassified according to the World Health Organization 2008 classification. Results: These 361 cases include 336 primary and 25 cases of lymphomas, where the involvement was secondary. Primary lymphomas consisted of 267 males (79.64%) and 68 females (20.24%) with a male:female ratio of 3.93:1. The mean age was 45 years (range 3-88). Diffuse large B-cell lymphoma (DLBCL) was the commonest subtype (222 cases; 66.71%), followed by low-grade marginal zone lymphoma of the mucosa associated lymphoid tissue (MALT) type (34 cases; 10.12%) and Burkitt's lymphoma (35 cases; 10.48%). The commonest site was stomach (180 cases; 53.57%), followed by small intestine (79 cases; 23.51%) and large intestine (68 cases; 20.23%), respectively. There were some uncommon types of GIT lymphomas documented during the study. Conclusion: In this largest retrospective single centre study from India, we establish that the pattern of distribution of primary GIT lymphomas (PGLs) in India is similar to the western literature in that the stomach is the commonest site of PGL and DLBCL is the commonest histological subtype. Immunoproliferative small intestinal disease cases were seen in this study, which is uncommon in the west.

Acute eosinophilic appendicitis and the significance of eosinophil - Edema lesion.

Eight cases of a histological entity that we name 'acute eosinophilic appendicitis' (AEA) are described and compared with classical acute appendicitis and appendices, which turned out to be normal after emergency appendicectomy. These 8 cases formed part of a dataset of 128 emergency appendicectomies, which also included 78 cases of classical acute appendicitis and 42 cases of normal appendix. The clinical and pathological variables were compared between these three. Acute eosinophilic appendicitis is characterized by acute presentation and a grossly inflamed appendix, but with absence of neutrophils in the muscle layer. The histological hallmark of the entity is eosinophil infiltration of the muscularis propria with accompanying edema separating muscle fibers (the Eosinophil - Edema lesion). The duration and prevalence of symptoms, the male female ratio and the gross maximum circumference of the appendix in these 8 cases resembled classical acute appendicitis rather than those cases in which the morphology of the appendix was normal. The Eosinophil-Edema lesion was also seen in many cases of classical appendicitis in non-suppurative areas where neutrophils were sparse or absent. We hypothesize that this lesion is an allergic response and is probably an early event in the evolution of acute phlegmonous appendicitis. Acute eosinophilic appendicitis may merely be those cases that do not proceed on to suppuration.