Iatrogenic immunodeficiency-associated lymphoproliferative disorders comprise a group of lymphoid neoplasms that are associated with an immunosuppressed state, either in the posttransplant period, or during the treatment of various autoimmune and rheumatologic disorders by immunomodulatory medications. Their morphologies vary widely but are generally classified according to the lymphomas that they most closely resemble. This group is strongly associated with infections by the Epstein-Barr virus as a result of impaired immune function in the immunosuppressed state. Although further classification may become necessary in the coming years, they are distinguished from lymphomas in immunocompetent hosts because reduction or cessation of immunosuppressive or immunomodulatory therapy can result in complete clinical remission.

Background: Cutaneous leishmaniasis (CL) is a parasitic cutaneous infection caused by Leishmania parasite. The histopathology is usually granulomatous in nature. Aims: The aim of the present study is to elucidate the histology of CL and evaluate the presence and the frequency of panniculitis among the affected patients. Settings and Design: Case series interventional study. Materials and Methods: Thirty-five patients with CL were diagnosed clinically between December-2012 and May-2013. Diagnostic confirmation established by smears, culture, and polymerase chain reaction (PCR). The histopathological assessment was carried out to study the general pathology and to look for the presence of panniculitis. Statistical Analysis Used: Simple statistics utilized via SPSS version 16.0 (SPSS, Inc., Chicago, USA). Results: Eighteen women and 17 men with CL were enrolled in the present work with a mean duration of their disease was 3 months. The results of the diagnostic tests were as follow: The smear was positive in 21 (60%) of cases, Leishman-Donovan (LD) bodies were seen in 7 (20%) patients, culture was positive in 24 (68%), and PCR was positive in 32 (91.4%) patients. The epidermal changes included acanthosis, pseudoepitheliomatous hyperplasia, ulceration, focal spongiosis, and interface dermatitis while the dermal changes were dependent on the spectrum of the disease, so in the ulcerative lesions there was lymphohistiocytic infiltration with foci of plasma cells and sometimes aggregate of LD bodies, whereas in the dry lesions the pathology is mainly of epithelioid granuloma. Panniculitis was seen in 16 (46%) cases as a diffuse lymphohistiocytic infiltration of both the septum and lobules of the subcutaneous layer of the skin. Conclusion: Panniculitis is an important feature of CL that must be differentiated from other diseases that can simulate CL such as chronic skin infections, Discoid lupus erythematosus, and cutaneous lymphoma.

Introduction: The mediastinum is the central portion of the thoracic cavity, limited by pleural cavities laterally, thoracic inlet superiorly, and the diaphragm inferiorly. Housing numerous organs, it is a veritable Pandora's box, within which various lesions may develop. This study was conducted to assess the epidemiologic profile, clinicoradiological features, cytological, and histopathological findings in patients presenting with mediastinal masses in a tertiary care hospital over a period of 3 years. Materials and Methods: This is a retrospective study of cases presenting with mediastinal masses attending the Cardiothoracic Surgery Department of Medical College, Kolkata between May 2011 and April 2014. Detailed history, physical, and radiological findings were noted. Fine needle aspiration cytology (FNAC) was performed when feasible. Following surgery, histopathological, and immunohistochemical (IHC) examinations of the specimens were undertaken. Results: Of the 22 cases included in our study, ten were anterior, seven middle, and five posterior mediastinal masses. Fifteen cases were male and seven were female. Thymic pathology was detected in seven cases, lymphoma in five, extragonadal germ cell tumor (GCT) in three, schwannoma and pericardial cyst in two cases each and neurofibroma, ganglioneuroma, and retrosternal thyroid in one case each. The age group of the patients for each diagnostic category was found to be of significance. FNAC was done in 15 cases. IHC was required for classification of lymphoma cases (CD45, CD15, CD30, CD20, CD3, Tdt, CD34, and Ki-67). Conclusion: This study reflects the incidence of different mediastinal masses in West Bengal with their clinicopathologic correlation.

Background and Aims: Varicose vein (VV) is an important cause of morbidity in the young and elderly population. Many studies of the Western country suggest that matrix metalloproteinases (MMPs) and tissue inhibitors of MMPs have a crucial role in the pathogenesis of VV, but limited work has been done in Indian population. The aim of this study is to study detailed histology of VV and to see the expression of MMP-1, MMP-9 and tissue inhibitor of matrix metalloproteinase-1 (TIMP-1). Materials and Methods: A total of 63 cases of VV and 10 control leg veins were included in this prospective study. Paraffin sections of VV were prepared. Hematoxylin and eosin (H and E), Masson trichrome and Verhoeff's staining were performed. Immunohistochemistry of VV was done with MMP-1, MMP-9, and TIMP-1 antibodies. Cytoplasmic expression of MMP-1, MMP-9 and TIMP-1 were graded as intense positive (++), weak/slight positive (+), and absent (−). Results: Focal intimal thickening (47.6%), increased medial thickening (73%) and fragmentation of elastin fibers (84.1%) were the major histological changes noted in H and E and special stained sections. MMP-1 expression increased in all layers of VV in 58 cases (92.1%) as compared to control veins. As compared to the control veins, intimal and adventitial expression of MMP-9 were increased in 31 (49.2%) and 40 (63.5%) cases, respectively. Expression of TIMP-1 was absent in both the varicose and the control veins. Conclusion: Increased expression of MMP-1 and MMP-9 suggests they have an important role in the pathogenesis of VV.

Background: This study is based on finding of an inexplicable artifact that was seen in the tissue received as periapical granuloma. Aim: To observe the histological appearance of different commonly implanted food particles and easily incorporated substances from a laboratory in the oral biopsy tissues. Materials and Methods: Various food particles such as wheat chapatti, beans, peas, pulses, and coriander leaves and substances such as a suture, cotton, and paper that can easily gain entry during biopsy and histotechnical procedures were intentionally introduced in the tissue specimens of a uterus from outside. Both light and polarizing microscopes were utilized to view them. Results: Different food particles and substances gave different appearances that could lead to misdiagnosis. Some of these also exhibited positive birefringence under the polarizing microscope. Conclusion: Knowledge and familiarity with probable foreign substances which can appear in tissues may help prevent misdiagnosis or erroneous diagnosis of biopsy specimens.

Introduction: The aim of this study was to assess whether epidermal growth factor receptor (EGFR) overexpression was a significant prognostic factor in clear cell renal cell carcinoma (CRCC) and whether its prognostic significance was affected by immunohistochemical expression patterns. Materials and Methods: Immunohistochemistry was performed on 100 cases of CRCC using an antibody against EGFR. Tumors were grouped by nuclear grade (NG) as low-NG (NG1, 2) or high NG (NG3, 4), and by pathological stage as localized (pT1, 2), or locally invasive (pT3, 4). Clinical disease was grouped by clinical stage as early stage (stage I, II), or late stage (stage III, IV). Evaluation of the EGFR overexpression was based on cytoplasmic (EGFR _Cyt ), and membranous (EGFR _Mem ) staining. Results: EGFR _Cyt correlated with high NG (P = 0.001), lymphovascular invasion (P = 0.028), regional lymph node involvement (P = 0.027), metastasis (P = 0.001), late stage (P = 0.003), cancer-specific death (P = 0.036), and was a predictor for disease-specific survival (P = 0.012) whereas EGFR _Mem correlated with only local invasion (P = 0.021) and perirenal invasion (P = 0.009) and did not show any correlation with cancer-specific death or disease specific survival. Conclusion: Our findings suggest that EGFR overexpression is an important prognostic factor in CRCC, and its prognostic value differs significantly with respect to the location of EGFR immunostaining. This prognostic difference may give direction on the management and treatment of CRCC patients.

Purpose: Diffuse large B-cell lymphoma (DLBCL) is an aggressive non-Hodgkin lymphoma with marked biologic heterogeneity. MYC and BCL2 rearrangements have been reported in a proportion of DLBCLs, where they may be associated with an adverse clinical outcome. The aim of this study was to determine the frequency of MYC and BCL2 translocations in DLBCL and assess the prognostic impact in DLBCL patients. Materials and Methods:   In the present study, we evaluated the expression patterns of CD 10, BCL6, and MUM 1 by immunohistochemistry in 121 cases with DLBCL in tissue microarray (TMA): 62 cases in germinal center B-cells (GCBs); and 59 cases in activated B-cells (ABCs) of which 60 were females and 61 were males. MYC and BCL2 rearrangements were investigated by interphase fluorescence in situ hybridization on TMAs in 97 DLBCLs. Result: MYC rearrangements were observed in 11 of 97 cases. There was no association with other clinical features, including age, sex, and nodal/extranodal disease. MYC rearrangement was associated with significantly worse overall survival (P < 0.01). BCL2 rearrangements were observed in 14 of 97 cases. There was no association with other clinical features including age and sex. BCL2 rearrangement had a worse outcome (P < 0.01). MYC and BCL2 rearrangements were observed in 3 of 97 cases with the age of  53 (female), 53, 63 years old, respectively, died in 24, 18, and 35 months after the diagnosis. Two cases had primary nodal and one case primary extranodal presentations. All these patients had stage IV disease. Conclusion: We concluded that C-MYC and BCL2 may contribute to aggressive transformation, and more mechanism-based therapy should be explored. Targeted therapies involving these rearrangements and its associated pathways may change the fate of DLBCLs. Analysis of MYC gene rearrangement along with BCL2 is critical in the identification of high-risk patients with poor prognosis.

Context: The genus Streptococcus consists of more than 65 species. The taxonomic classification of these members is not well-defined. Among the viridans group streptococci (VGS), there are major taxonomic changes by the addition of many new species; whereas, most of the new strains are of animal origin and only a few have been reported to be isolated from humans. Rare and unusual species of VGS such as Streptococcus thoraltensis, S. pluranimalium and S. hyointestinalis are normally associated with different animals. Their isolation from human being is not yet reported. Aim: To find out the rare and unusual species of viridans group streptococci from human oral cavity. Settings and Design: A case-controlled study carried out at hospital-based dental services in a tertiary care hospital. Materials and Methods: Subgingival plaque samples of the tooth were collected from 80 patients (34 with periodontitis and 46 without periodontitis) undergoing tooth extraction. Cultures were subcultured onto special media such as Tryptone Soya blood Agar supplemented with strepto supplement and Mutans-Sanguis Agar. Identification of strains and antimicrobial susceptibilities were measured as minimum inhibitory concentration using Vitek 2 (BioMérieux, Paris, France) automated system. Results: We have identified three strains of VGS - S. thoraltensis, S. pluranimalium and S. hyointestinalis from subgingival plaque samples from patients with periodontitis. S. thoraltensis and S. pluranimalium were found to be resistant to most of the antibiotics. Conclusions: To the best of our knowledge, this is the first report of isolation of these rare and unusual strains from the human oral cavity.

Background: Several enterococcal species are increasingly being reported from clinical infections, besides the major species. Aim: This study was undertaken to determine the prevalence of unusual enterococcal species and their antimicrobial susceptibility patterns, virulence factors, and molecular characterization. Study Design and Settings: The study was conducted in Department of Microbiology and associated Tertiary Care University Hospital in North India. Materials and Methods: Enterococcal isolates were collected for a period of 2 years from clinical specimens. Identification and elaborate phenotypic characterization was done biochemically. All the isolates were tested by Kirby-Bauer disc diffusion method and breakpoint minimum inhibitory concentration for susceptibility against standard antibiotics. Screening for vancomycin-resistant enterococci (VRE), high-level aminoglycoside resistance was done on brain heart infusion agar incorporated with 6 μg/ml vancomycin, 500 μg/ml gentamicin, and 2000 μg/ml streptomycin, respectively. VRE isolates were tested for the presence of vanA, vanB, and vanC genes and high-level gentamicin resistant (HLGR) isolates for aac-6'- aph-2' gene by polymerase chain reaction (PCR). Hemolysin and gelatinase production, hemagglutination and biofilm formation were detected along with asa1, gelE, esp, hyl, and cylA genes by multiplex PCR. Results: Of 403 enterococci, 93 (23.07%) isolates were identified as unusual species and atypical variants. Resistance of 52.68%, 46.23%, 44.08%, and 6.45% for ampicillin, ciprofloxacin, high strength gentamicin, and vancomycin, respectively were noted. Presence of vanC gene in Enterococcus gallinarum and Enterococcus casseliflavus isolates and vanA gene in Enterococcus durans and Enterococcus hirae and aac-6'- aph-2'' gene was found in 33.14% (14/41) of the HLGR isolates. The most frequent virulence factor was biofilm production. Only a few isolates harbored asa1 (2), gelE (9), and hyl (3) genes. Conclusion: Considerable prevalence of pathogenic unusual species of enterococci was seen along with their emerging drug resistance and virulence. Complete identification and routine speciation is essential to limit their emergence as major species in near future.

Paragonimiasis is an infectious disease caused by Trematodes of the genus Paragonimus that is endemic in Asia, Africa, and South America. Most patients with paragonimiasis are cured by standard praziquantel treatment. However, several cases have been reported to have unsatisfactory responses to the standard praziquantel treatment. To probe the clinical characteristics, possible cause, and management of the paragonimiasis individuals improved by multiple therapies, we present a 12-year-old Chinese boy, who was infected with Paragonimus accompanied by arachnoid cyst involvement, as not having typical clinical symptoms, but repeatedly presenting with migrated lesions between the lung and pleura. He responded to treatment with 3 cycles of praziquantel and 1 cycle of albendazole.

As the incidence of end-stage renal disease (ESRD) is rapidly increasing, the demand for dialysis and transplantation has dramatically increased, which has led to concerns about the availability and equitable allocation of kidneys for transplantation. The distribution of HLA-A, B and DR alleles in 148 renal transplant recipients and 191 live related prospective donors from 2009 to 2010 were analyzed. Allele frequencies and haplotype frequencies were calculated in recipients and donors. The prospective donors were further analyzed on the basis of their relationship to the patients and according to the sex ratio. A significant female preponderance was noted in the prospective donor population, most of whom were either siblings or parents of the recipients. On the contrary, the recipient population predominantly comprised of males. The most frequent HLA-A, HLA-B, HLA-DRB1 alleles in renal transplant patients were HLA-A*11, A*02, A*01, A*24; HLA-B*35, B*40, B*44, B*15, B*52, and HLA-DRB1*15, DRB1*07, DRB1*13, DRB1*11 respectively. The most frequent HLA-A, HLA-B, HLA-DRB1 alleles in prospective donors were HLA-A*02, A*11, A*33, A*24; HLA-B*35, B*44, B*40, B*15 and HLA-DRB1*15, DRB1*07, DRB1*11, DRB1*13 respectively. A*11-B*35, A*02-DRB1*15, B*40-DRB1*15 were the most common HLA A-B , HLA A-DR, HLA B-DR haplotypes respectively in renal transplant patients, whereas, A*11-B*35, A*11-DRB1*15, B*44-DRB1*07 were the most common haplotypes in renal donors. In three locus haplotype, HLA-A*02-B*40-DRB1*15 was the most frequent haplotype in patients, whereas, in prospective renal donors HLA-A*33-B*44-DRB1*07 was the most frequent haplotype.

Current influenza A(H1N1)pdm09 strain severely involved many parts of the country. The study was conducted to analyze the clinicoepidemiological trend of influenza A(H1N1)pdm09 cases from October 2014 to March 2015. Samples processing was done as per the Center for Disease Control guidelines. A total of 333 specimens were processed out of which influenza A(H1N1)pdm09 constituted 24% (81) of total, 5% (18) cases were seasonal influenza A virus strains. Mean age group involved was 49 years with case fatality rate of 20%. Patients died were 63% males and 44% had comorbidities, and among them, 38% patients died within 24 h of hospitalization. The mean age of comorbid patients who died was 59 years; whereas the mean age of patients died having no co-morbidities was 41 years (P < 0.005). Mortality was seen among 81% (13) of patients who were on ventilator support. Added mortality in specific human group demands continuous surveillance monitoring followed by the detection of mutation, even in susceptible animal population.

Helicobacter pylori (HP) is causally associated with peptic ulcer disease and gastric carcinoma. Determination of the prevalence of HP infection in dyspepsia patients' in particular geographical area is imperative for the appropriate management of dyspepsia. HP antigen detection in stool is a noninvasive diagnostic test of HP infection. This prospective study was conducted to find out the prevalence of HP infection based on stool antigen testing in dyspeptic patients who had also undergone upper gastrointestinal (GI) endoscopy. This study highlights the high prevalence of HP infection in dyspeptic Indian patients, particularly males, and emphasizes the growing importance of the bacterium causing infection among children. We also found HP stool antigen testing to be superior to upper GI endoscopy for detecting HP infection. Hence, we recommend initial testing for HP stool antigen in dyspeptic patients before initiating treatment and before carrying out any invasive procedure such as endoscopy.

Liposarcomas are extremely rare in the mediastinum. Patients usually present late due to the compressive effect of the tumor on the adjacent structures. Severity of the symptoms depend mainly on the size of the tumor and the structure it infiltrates. Well differentiated slow growing liposarcomas are the most common ones in the mediastinum followed by dedifferentiated and poorly differentiated ones. These tumors have bad prognosis because of incomplete surgical excision due to its inaccessible location. Hence these patients should be kept under close follow up because of high recurrent rates. Here we are presenting a rare case of anterior mediastinal sclerosing liposarcoma in a 77 year old male.

Mixed gonadal dysgenesis (MGD) is a rare disorder of sexual development (DSD). A seven-year-old child with vagina and an elongated clitoris looking like a penile part of male external genitalia, presented in the Paediatric Surgery Department. Ultrasound of pelvis revealed a streak ovary on the right side and ovotestis on the left side. Uterus was not visualized. Karyotyping revealed 46, XY/45, XO genotype. Laparoscopic gonadectomy with vaginoplasty and the clitoral reduction was performed. A thorough histopathological examination of the specimen revealed structure of ovotestis-consisting of the ovary with Graafian follicles, fallopian tube, infantile testis with Leydig and Sertoli cells, epididymis, vas deference, and pampiniform plexus. Presence of Graafian follicle in the ovary, Leydig and Sertoli cells in the testis, at this age makes this case extremely interesting. The child was rehabilitated to lead her life as a girl.

Collagen type III is a normal component of interstitium and blood vessels. Collagenofibrotic glomerulopathy (CG) and nail patella syndrome (NPS) are the diseases of abnormal type III collagen deposition. In spite of these curved frayed structures with a periodicity of 45-60 nm are deposited in subendothelium and mesangium in CG, they are found only in the basement membrane in NPS. The clinical features of CG are confined to the kidney, NPS has associated extra-renal manifestations. Electron microscopy is essential to make the renal diagnosis in both these rare diseases. Both the entities considered to be systemic diseases evidence to suggest similar deposition in other organs, understanding etiopathogenesis and disease progression await research.

Angiosarcomas are rare soft tissue neoplasms accounting for <2% of all sarcomas. Rare cases have been reported in other sites like breast, spleen, liver, bone and bladder. This is a case report of an angiosarcoma of the bladder in a 65 year old lady who came with complaints of haematuria. Ultrasonography and computerized tomography revealed a bladder tumour with calcification, which on cystoscopy also showed a calcified mass. The patient had a past history of carcinoma cervix for which she had undergone surgery and radiotherapy, 22 years ago. Biopsy of the bladder mass showed features of a high grade epithelioid sarcoma with extensive osseous metaplasia. On immune stains,the tumour was positive for CD31 and vimentin and negative for cytokeratin, desmin and CD34. Until now, only sixteen cases of angiosarcoma of the bladder have been reported in english literature, of which 6 occurred secondary to radiation. Our case is the first to occur after 22 years of radiotherapy and have extensive osseous metaplasia.

Three histological subtypes of lymphomatoid papulosis (LyP), type A (histiocytic), type B (mycosis fungoides like) and type C (anaplastic large cell lymphoma like) are well recognized. Two new histological variants, type D (simulating an aggressive epidermotropic cytotoxic lymphoma) and type E (angioinvasive type) has been described recently. We describe a 27-year-old man presented with a history of asymptomatic erythematous papules on both upper and lower limbs noted since 10 years of age. There were no systemic symptoms. Biopsy revealed an atypical dermal lymphoid infiltrate with epidermotropism, and the immunohistochemical markers showed a diffuse positivity for CD3, CD8, CD56, T1A and granzyme B with the focal positivity of CD30. All other relevant tests were normal. In this case report of a recently described delineated variant of LyP we emphasize the indolent course of this entity although the histology would suggest a more aggressive disease.

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40-year-old male who presented to our hospital with pyrexia of unknown origin and rash of 3 weeks duration. All his serological investigations and imaging studies were unremarkable. He was fulfilling clinical and laboratory criteria as per Yamaguchi for AOSD and was managed for the same. Our patient did not respond well to the treatment, had a downhill course, and succumbed to his illness. Autopsy confirmed myocarditis and florid bone marrow reactive hemophagocytosis as the cause of his death.

Primary leiomyomas are rare benign tumors of the lung and only 25 cases have been documented in children, most of which are endobronchial. Leiomyomas are benign smooth muscle neoplasms, usually diagnosed on morphological characteristics. However, immunohistochemistry plays a crucial role in the lineage differentiation when these are encountered at unusual sites or with unexpected morphological features. We report a case of endobronchial tumor of a 13-year-old male child who presented with a dry cough and hemoptysis. A mass lesion in the right main bronchus was detected by bronchoscopy and contrast-enhanced computed tomography. On histopathological examination of the resected specimen, a diagnosis of myxoid leiomyoma was made. To the best of our knowledge, this is the first case of myxoid leiomyoma to be reported in the respiratory tract. This case also demonstrates the need for a high index of suspicion and the role of immunomarkers in the diagnosis of such challenging cases.

Granular cell tumor (GCT) is a rare benign mesenchymal tumor that uncommonly occurs in the lung and tracheobronchial tree. Small cell carcinoma of lung is a centrally located malignant neoplasm that commonly occurs in elderly smokers. Concomitant existence of both the neoplasm in lung is extremely rare with only one reported case in the literature. Few rare combinations of GCT with other primary bronchogenic carcinomas have also been reported. Clinical symptoms depend upon the site and size of the tumor. Definitive diagnosis is by histopathological and proper immunohistochemical analysis. Identification of this entity is important as treatment requires individual therapy protocols that depend on the presence of metastasis, location of the tumors, and type of bronchogenic carcinoma.

Inflammatory myofibroblastic tumors (IMTs) are uncommon benign neoplasms in the abdomen, and urachal involvement is very rare. IMT of the urachus is usually mistaken clinically and radiologically for a malignant tumor. The tumor has spindle cell proliferation with a prominent inflammatory infiltrate and myxoid degeneration favoring other designations such as inflammatory pseudotumor and fibromyxoid pseudotumor. Complete excision is the treatment of choice. The case documented here was in a 50-year-old male, with an abdominal mass diagnosed on computed tomography scan as adenocarcinoma of urachus infiltrating the bladder. Histopathology showed a benign spindle cell tumor invading bladder muscle. Immunohistochemistry aided in diagnosing an anaplastic lymphoma kinase (ALK)-1 positive IMT. Approximately, 50% of the IMTs show ALK positivity, and though when present, it indicates neoplastic nature of the spindle cells, the pathogenesis of IMT is still elusive. The urachal IMTs need documentation in literature for their rarity and diagnostic dilemma posed by them.

Lipofibromatous hamartoma (LFH) is a rare, benign fibrofatty tumor composed of a proliferation of mature adipocytes within peripheral nerves, which form a palpable neurogenic mass. It affects the median nerve in 66-80% of cases, causing pain and sensory and motor deficits in the affected nerve distribution. Patients typically present with gradually enlarging nontender lesions in the distribution of the affected nerve. The lesion is also seen to be associated with macrodactyly. The pathophysiology of LFH is unknown. Treatment of LFH is based on symptoms of the condition. Histopathology is characteristic. We present a case of young male diagnosed as lipofibromatous hamartoma of the median nerve involving the right index finger. The case is presented due to its rarity.

Haemoglobin (Hb) Agenogi is clinically asymptomatic, rare β-globin chain variant characterized by a substitution of glutamic acid by lysine at position 90 of β-chain. It elutes in the C-window on high-performance liquid chromatography (HPLC). We report a 10-year-old male with easy fatigability, lethargy, pallor, and mild splenomegaly. Hematological parameters revealed microcytic hypochromic anemia and mildly raised red blood cells count, suggestive of thalassemia trait. On HPLC, a predominant peak was observed in the C-window (82.6%) along with raised HbA ₂ level (9.3%). Based on these findings, a possibility of HbC disease/β-thalassemia trait doubly heterozygous was considered. Family studies were advised. HPLC findings in father were suggestive of β-thalassemia trait, while both his mother and brother had an abnormal peak in the C-window of 42.7% and 40.8%, respectively, with elevated HbA ₂ values of 5% and 4.9%, respectively. Direct DNA sequencing revealed intervening sequences 1-5 (G ; C) in father, confirming β-thalassemia trait. His mother and brother had heterozygous gene mutation at codon 90 of β-globin chain (G ; A) suggestive of Hb Agenogi. The child carried mutations for both β-thalassemia trait as well as Hb Agenogi.

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.

The blast crisis of chronic myelogenous leukemia (CML) can be hematological or extramedullary. About 25% of these cases fulfill the criteria for mixed phenotype acute leukemia. We here report a case of a second blast crisis of CML which was extramedullary and was immunophenotypically bilineage T/myeloid.

Griscelli syndrome (GS) is a rare autosomal recessive immunodeficiency disorder in which the affected children present with characteristic silvery-white hairs. The hair microscopy of these children is characteristic and is helpful in differentiating GS from Chediak-Higashi syndrome which also presents with immunodeficiency and silver hairs. We report a 17-month-old boy with GS type 2 who presented with severe anemia. Bone marrow examination of the child suggested parvovirus B19 as the cause of severe anemia, which was later confirmed by DNA polymerase chain reaction.

Griscelli syndrome (GS) is a rare autosomal recessive disorder caused by mutation in the MYO5A (GS1), RAB27A (GS2), and MLPH (GS3) genes, characterized by a common feature, partial albinism. The common variant of three, GS type 2, in addition, shows primary immunodeficiency which leads to recurrent infections and hemophagocytic lymphohistiocytosis. We, herewith, describe a case of GS type 2, in a 4-year-old male child who presented with chronic and recurrent fever, lymphadenopathy, hepatosplenomegaly, and secondary neurological deterioration; highlighting the cytological and histopathological features of lymph nodes. Hair shaft examination of the child confirmed the diagnosis.

Keratomycosis or fungal infections of cornea are common causes of ocular morbidity particularly in developing countries and in tropical climate. Traumatic inoculation is the predominant predisposing factor for this condition. Most of the cases are caused by filamentous fungi. Blastoschizomyces capitatus is one of the emerging fungal agents causing infection in different organ systems particularly in immunocompromised individuals. Barring one case of keratitis and melting of corneal graft there is no report of keratomycosis by B. capitatus. Here we present a case of keratomycosis caused by B. capitatus, which is the first such case reported from India.

Sphingomonas paucimobilis , a yellow-pigmented, aerobic, glucose nonfermenting, Gram-negative bacilli is a rare cause of human infection. It was first discovered as an infective agent in humans in 1977 and named Pseudomonas paucimobilis. It was renamed as S. paucimobilis in 1990 in accordance with phylogenetic data. S. paucimobilis is an aerobic bacterium found in soil and water; it is a rare cause of healthcare associated infections. S. paucimobilis can cause infections in healthy as well as immunocompromised individuals. At first, its colony looks like Gram-positive bacilli colony, so by mistake it is discarded as contaminants. S. paucimobilis is an emerging pathogen and it should not be discarded as contaminants. Here, we report a case of S. paucimobilis bacteremia in a neonate who presented with respiratory distress.