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Unlike in the west, breast cancer in India presents more than a decade earlier, predominantly in stage III, and with an unusually large percentage of poor-prognosis triple-negative subtype. Vitamin D deficiency prevails in epidemic proportions in India. Cross-sectional observational study to correlate serum vitamin D levels with molecular subtypes of breast cancer. In fifty breast cancer patients, core needle biopsy was used for receptor status and molecular subtyping. Serum vitamin D was documented prior to treatment. Serum vitamin D levels were correlated with molecular subtypes of breast cancer. The mean age was 45.68 years. None were in stage 1, while 48% were in stages 2 and 3 each. On biopsy, 90% were ductal carcinoma. The largest group of patients was triple negative (34%). In triple-negative subtype, majority (71%) were stage 3. Fifty percent of stage 3 patients were triple-negative subtype. The mean serum vitamin D levels were 15.78 SD 7.34 ng/ml. The lowest mean serum vitamin D levels were seen in triple-negative subtype, i.e., 11.49 SD 4.17. The difference was statistically significant among the subtypes. Vitamin D deficiency (present in 96% of our patients) has a significant association with triple-negative breast cancer (associated with worst prognosis), as compared to other molecular subtypes, suggesting vitamin D deficiency to be a likely significant risk factor for the development of breast cancer, particularly triple-negative subtype which is present in unusually large proportion in India. Identification of vitamin D deficiency as an important (and easily modifiable) risk factor for breast cancer could help define effective preventive strategies.

Background Histoplasmosis is an endemic granulomatous fungal infection which rarely infects the larynx; less than 100 cases have been reported in medical literature till date. Case presentation A 6-year-old female child, a diagnosed case of B cell acute lymphoblastic leukemia since past 2 years and on chemotherapy, presented with hoarseness and dysphagia since past 2 months. Direct laryngoscopy revealed granular appearance of posterior pharyngeal wall and the supra-glottic larynx. Histopathological examination and bone marrow aspiration confirmed the diagnosis of disseminated histoplasmosis. Conclusions Pharyngo-laryngeal histoplasmosis should be considered in the differential diagnosis of an immunocompromised patient presenting with persistent hoarseness.

Beta thalassemia major is associated with a subclinical hypercoagulable state. Endothelial activation markers like soluble Intercellular adhesion molecule (sICAM-1) and E-selectin have been implicated in the pathogenesis of endothelial dysfunction and hemostatic alterations. In this study we aimed to study serum levels of sICAM-1 and E-selectin in polytransfused children with β thalassemia major and their association with serum ferritin and D-dimer levels. Sixty-two polytransfused β-thalassemia major children aged between 5 and 17 years and 26 age and gender matched healthy controls were enrolled in the study. Complete blood count with peripheral smear, liver function tests, serum ferritin, coagulation tests [PT, APTT, D-dimer] and endothelial activation marker tests [ICAM-1 and E-selectin] were performed. PT, APTT and D-dimer levels were significantly higher in beta-thalassemia major patients than in control group (p = 0.003, p

The pathogenesis of hypercoagulability in HIV infection is multifactorial and usually more than one factor is responsible for a thromboembolic episode. The present study was conducted to evaluate the effect of HIV infection and antiretroviral therapy on various coagulation parameters in paediatric patients. Forty two newly diagnosed paediatric patients with HIV infection who were enrolled at the Anti-Retro viral Therapy (ART) centre of Kalawati Saran Children’s Hospital were included in the study. The patients were grouped into 4 clinical stages according to the WHO clinical staging of HIV disease. Coagulation tests [PT, aPTT, fibrinogen, D-Dimer and coagulation inhibitors i.e. Protein C (PC), Protein S (PS) and antithrombin III (AT III), Lupus anticoagulant (LA) and Anti phospholipid antibody (APLA)] were performed in all the patients at the time of diagnosis and repeated after 6 months. All the patients were started on antiretroviral therapy within 2 months of their diagnosis. At the time of diagnosis, prolonged PT and aPTT were observed in 30.9% and 23% of the cases respectively. Hyperfibinogenemia was seen in 11.9% of patients. D-Dimer was raised in 83.3% of patients. PS, PC & AT activities were reduced in 90.4%, 42.8% & 11.9% of cases respectively. A reduction in the PC and AT activity was seen from clinical stage 1 to 4, but the change was not statistically significant. On follow up after 6 months, a statistically significant reduction in the level of fibrinogen and D-Dimer was seen. Even though there was improvement in the activity of all the coagulation inhibitor after 6 months, statistically significant improvement was seen only for PS. The current study shows that HIV produces a hypercoagulable state in children. Raised d-dimer level and deficiency of natural anticoagulants contribute to the thrombophilic state.

Autocrine and paracrine loop involving vascular endothelial growth factor (VEGF) and its receptor have been described in haematological malignancies. However, scarce literature is present on angiogenesis in paediatric acute lymphoblastic leukemia (ALL) with studies showing controversial results. The aim was to study serum levels of VEGF and its receptors in paediatric ALL at the time of diagnosis and at the end of induction phase and to compare these levels with clinico-haematological parameters in these patients. Serum VEGF, VEGFR-1 and VEGFR-2 levels were measured by enzyme-linked immunoabsorbant assay at diagnosis (day 0) and at the end of induction phase (day 35) in 30 newly diagnosed paediatric ALL patients and in 10 healthy controls. Median s-VEGF was significantly lower at day 0 as compared to day 35 (196.15 vs. 606.75 pg/ml: p

Granular cell tumor (GCT) of the oral cavity is rare and so is the involvement of the eye, orbit, and ocular adnexa. A 65-year-old male developed a posttraumatic ulceroproliferative mass over his left cheek for the past 1 year. The mass involved the periorbital region with accompanying blood-stained purulent discharge from multiple sinus tracts over this lesion for the past 6 months. Radiographs of the orbit suggested chronic osteomyelitis. The lesion was not responsive to treatment with antibiotics. Enlarged submandibular lymph node demonstrated reactive lymphadenitis on cytological examination. However, computed tomography scan of the paranasal sinus (PNS) revealed possibly a malignant mass extending into the maxillary sinus and left extraconal space. Surprisingly, histopathological examination and immunohistochemistry from a growth involving the left upper retromolar region that extended up to the midline and periorbital region suggested a diagnosis of GCT. This unusual and new presentation of GCT is not well known to the dentists and also to the ophthalmologists. It is imperative to examine anatomically neighboring structures, especially the eye, nasal cavity, PNSs, and oval cavity among other structures in an underlying pathology in either of these sites.

Becker’s nevus (BN) is an acquired unilateral hyperpigmented hairy macule, whereas morphea is a chronic connective tissue disease of unknown etiology, characterized by skin thickening with increased quantities of collagen in the indurated lesion, usually involving the upper trunk and proximal extremity. The occurrence of both disease in the same anatomical site and individual was not reported previously. We report this rare case in a 17-year-old, Indian girl and was diagnosed after clinical histopathology and dermoscopic correlation. Morphea can be an interesting incidental occurrence within a large plaque of BN.

Background: Aplastic anemia is a result of complex immune mediated marrow injury. As activated T cells play a pivotal role in immune response, study of cytokine profile in patients of aplastic anaemia (AA) will be of use in understanding the initiation and propagation of the disease. Methods: Thirty children with AA between ages 6 months to 18 years were included along with age & sex matched controls. Complete blood count (CBC) and bone marrow examination was done. They were grouped as Very severe aplastic anemia (VSAA), Severe aplastic anemia (SAA) and Non severe aplastic anemia (NSAA) as per standard guidelines. Intracytoplasmic expression of Type 1 cytokine (IFNγ) and Type 2 cytokine (IL4) was determined by Flow cytometry (FCM). CD8 and CD4 cell counts were also determined by FCM. Result: CD8 cell count was higher than the CD4 counts with reversal of CD4:CD8 ratio in all patients. Mean intracytoplasmic IFNγ expression in CD8 and CD4 cells was higher in cases than in controls (p

The incidence of abdominal cysts in infants is 1 in 500 to 1 in 1,000 live births. Among the ovarian cysts in infants, serous cystadenoma is extremely rare with only few reported cases in the literature. Here, we report a case of a giant neonatal ovarian serous cystadenoma treated with laparoscopic cystectomy and confirmed by histopathological examination.A 27-year-old delivered a female baby with uneventful caesarean section at full term. The antenatal ultrasonography (USG) in third trimester had showed an abdominal cyst in the left side of the abdomen. Postnatal USG was suggestive of omental cyst. A contrast-enhanced computed tomography scan of the neonate showed a large cyst occupying the entire abdomen. On laparoscopic evaluation, a cystic mass filled with 500 mL of clear yellow fluid was seen in the left pelvic fossa. Left ovary could not be visualized separately. The right ovary, fallopian tubes, and uterus were normal. The entire cyst was removed and sent for histopathological examination. On gross examination, a unilocular cyst measuring 10×8×6.5 cm with a wall thickness of 0.2 cm was noted. On microscopic examination, the histomorphological features were consistent with serous cystadenoma of the ovary. There is a paucity of literature regarding pathological diagnosis of such cases and hence we report one such case.

Objective To determine the appropriateness of using MCV/MCH as screening test for beta-thalassemia trait in the present population and also to find the most appropriate cutoff for optimum sensitivity of these indices. Methods It was an analytical, observational and cross-sectional study. Complete blood count followed by high-performance liquid chromatography (HPLC) was performed. The MCV and MCH levels were noted in cases and controls. Results Thalassemia trait was found in 66 out of 1300 antenatal women with anemia. The MCV and MCH were significantly low in cases (p = 0.0001). MCV had a better AUC (0.650) than MCH (0.635). The most suitable cutoff value of MCV was calculated as 72 fl (sensitivity-63.7%, specificity-68.3%, PPV-9.7%, LR-2.0) and that for MCH was 24 pg (sensitivity-63.6%, specificity-59.4%, PPV-7.7%, LR-1.5) using Youden's index. When MCH (cutoff of 28 pg) and MCV were combined (cutoff of 74 fl), the sensitivity and specificity were 95% and 16%, respectively. Conclusion The sensitivity and specificity of MCV and MCH alone had low detection rate when used in combination had high sensitivity but the specificity was low; therefore, HPLC should be the preferred screening test for beta-thalassemia in Indian women.

Sarcomatoid urothelial carcinomas (SUCs) are characterized by intimately mixed malignant epithelial and malignant mesenchymal components. We present a case of a multifocal SUC in the left pelvicalyceal system of the kidney and the left ureter. SUC although rare should be included in the differential diagnosis of multifocal tumors of the renal pelvis and ureter. Immunohistochemistry is essential to confirm the diagnosis and rule out other differentials including pure sarcoma, sarcomatoid variant of renal cell carcinoma, and pseudosarcomatous mesenchymal proliferation.

Breast hamartomas (BH) or Fibroadenolipoma are extremely rare breast lesions. They are benign, focal malformations that resemble a neoplasm in the tissue of its origin. We describe a case of a 48 year old woman with bilateral breast lumps for the past 18 months and 6 months which were gradually increasing in size. FNAC smears revealed only clusters of benign ductal epithelial cells. Lumps were excised and histopathology revealed predominantly stromal smooth muscle bundles (proved by immunohistochemistry) along with scattered intact terminal duct-lobular units, dilated ducts and entrapped islands of mature fibroadipose tissue. A diagnosis of Bilateral Myoid hamartoma of breast was given. To the best of our knowledge till date no case of bilateral MH of breast has been reported in the literature. We report the first case of bilateral MBH highlighting its morpho-immunophenotypic features.

Castleman disease is a rare clinicopathological disorder associated with lymphoproliferation. We report the case of a 54 year old gentleman initially presenting with dysphagia followed by bilateral cervical lymphadenopathy, and later diagnosed as multicentric Castleman disease. To the best of our knowledge no case of Castleman disease presenting as dysphagia along with neck swelling has been previously described in the literature. This case report highlights a very unusual presentation of this rare disease. It also provides insight into the fact that because of the lack of any specific presenting symptoms and distinguishing radiographic features, an accurate histopathologic diagnosis and careful staging become crucial to planning treatment for this disease. DOI: http://dx.doi.org/10.3329/bjo.v19i1.12041 Bangladesh J Otorhinolaryngol 2013; 19(1): 53-57

BACKGROUND Anemia of chronic disease (ACD) and iron deficiency anemia (IDA) are the two most prevalent forms of anemia having interrelated characteristics. Hepcidin, a newly introduced biomarker for assessment of iron status, is a homeostatic regulator of iron metabolism. We investigated the role of hepcidin and other conventional iron parameters to assess iron status among children with ACD and IDA. We also identified children with ACD who developed iron deficiency (ID). METHODS The study was undertaken in anemic children with 30 cases each of ACD and IDA along with 30 age and sex-matched controls. The ACD cases were subdivided into pure ACD and ACD with coexistent ID. All cases were subjected to following tests: complete blood count with peripheral smear, serum C-reactive protein, serum interleukin-6, iron studies, serum soluble transferrin receptor (sTfR), and serum hepcidin. RESULTS The mean serum hepcidin concentration was significantly increased in pure ACD patients (143.85±42.76 ng/mL) as compared to those in IDA patients (6.01±2.83 ng/mL, P < 0.001) and controls (24.96±9.09 ng/mL, P

Neutral lipid storage disease is a nonlysosomal multisystemic triglyceride storage disease. It is characterized by leukocyte vacuolization (Jordans’ anomaly), variable systemic involvement, and ichthyosis. Two of our patients presented with congenital ichthyosis. Lipid vacuoles were demonstrated in granulocytes and monocytes and in basal keratinocytes on skin biopsy. They were diagnosed as Chanarin Dorfman syndrome. In contrast to these cases, the third case presented with progressive symmetric erythrokeratoderma without ichthyosis. Lipid vacuoles were demonstrated in blood cells on peripheral smear and in basal keratinocytes. Only screening of peripheral smear led to the correct diagnosis.

Intraoral molluscum contagiosum is an extremely rare clinical entity with only seven cases reported till date in the world medical literature. This clinical record documents the first case of the said lesion in tongue in an immunocompetent female of 35 years old, who was treated surgically. In addition this case report also reviews the scant medical literature on the cited subject. With this case, we illustrate a rare entity that present in an extremely rare manner.

Authors present a rare case of primary ovarian leiomyoma in a young female with premature ovarian failure. A 39-year-old female with premature ovarian failure presented with large unilateral adnexal mass, with mildly elevated CA-125 levels, underwent staging laparotomy where the left ovarian mass along with tubes was removed. Contralateral ovary and uterus were preserved. Final diagnosis was Primary ovarian leiomyoma on histopathology and immunohistochemical analysis. Preoperative diagnosis is difficult with solid ovarian tumors. In presence of mildly elevated CA-125 and negative tumor markers, ovarian leiomyoma must be considered as a differential diagnosis and conservative surgery be planned as these patients are desirous of future fertility options. Definitive diagnosis is confirmed by histopathology and immunohistochemical analysis.

To study plasma levels of Thrombin activable fibrinolysis inhibitor (TAFI) in children with β-thalassemia major. Fifty β-thalassemia major patients, 1.4 to 17 y of age, with number of transfusions received varying from 21 to 162 were selected at random and complete blood count (CBC), coagulation parameters [Prothrombin time (PT), Activated partial thromboplastin time (aPTT), fibrinogen, D-dimer, protein C, protein S, antithrombin, Tissue plasminogen activator (t-PA), Plasminogen activator inhibitor (PAI-1)] and TAFI were performed. PT and aPTT were prolonged in 18 % and 30 % of cases respectively. Reduced activity of Protein C (PC) was observed in 50 % of cases and Protein S (PS) was reduced in 54 % of cases. t-PA levels were significantly higher in cases. TAFI levels were 17.24 ± 4.05 ng/ml which were significantly higher than the control group (15.01 ± 3.28; p = 0.003) No significant correlation of TAFI was observed with Hb, platelet counts, liver enzymes, serum ferritin, PC, PS, D-dimer, t-PA or PAI-1. There is an ongoing subclinical activation of coagulation cascade and fibrinolytic system in thalassemia major (TM) patients. Higher levels of TAFI in the present study with no significant correlation with other parameters were noted, thus pointing out to its independent role in contribution to hypercoagulable state in thalassemia. TAFI serves as a link between two limbs of hemostasis, with its higher levels promoting inhibition of fibrinolytic system and thus promoting a hypercoagulable state. Performing TAFI levels in thalassemic patients could help to detect the early coagulopathy in these patients and hence these patients can be closely monitored for any evidence of thrombosis.

To evaluate the coagulation parameters at the time of diagnosis in pediatric acute lymphoblastic leukemia (ALL) patients. A total of 65 newly diagnosed ALL patients upto 18 y of age along with 30 age and sex matched controls were included in the study. Coagulation tests including Prothrombin Time (PT), Activated Partial Thromboplastin Time (APTT), Fibrinogen (FBG) assay, D-dimer (D-DI) assay, Coagulation inhibitor levels and tests for fibrinolysis were performed. At baseline, APTT of the patients was significantly prolonged (p 0.033), but PT and fibrinogen were comparable in the two groups. Protein C (PC) and Protein S (PS) were both significantly reduced in the cases, while antithrombin was comparable to control values (p

To compare the levels of IgG and IgM anti beta-2 glycoprotein I antibodies and IgG and IgM anti prothrombin antibodies among women with unexplained recurrent pregnancy losses and women with at least 2 live issues. To compare the prevalence of newer anti beta-2 glycoprotein I & anti prothrombin antibodies with conventional Lupus anticoagulant & anticardiolipin antibodies. 50 women with recurrent pregnancy losses & 50 matched controls were evaluated for the presence of: Lupus anticoagulant—screened by LA sensitive aPTT& DRVV and confirmatory Staclot Assay. ELISA kits were used for detecting IgG & IgM anticardiolipin, anti beta-2 glycoprotein I & anti prothrombin antibodies. 11/50 (22 %) women in study group and none in control group had circulating antiphospholipid antibodies. 2 cases (4 %) had lupus anticoagulant. 1 case (2 %) had anticardiolipin antibody & 6 cases (12 %) were positive for anti beta-2 Glycoprotein I antibody (p value = 0.027). 3 cases (6 %) had anti prothrombin antibody. All were mutually exclusive except for one. Women with recurrent pregnancy losses should be tested for anti beta-2 Glycoprotein I antibodies & anti prothrombin antibodies in addition to conventional lupus anticoagulant and anticardiolipin antibodies. This approach can decrease the incidence of SNAP (seronegative antiphospholipid syndrome) cases while establishing the true prevalence of antiphospholipid syndrome.

An 18-year old girl with segmental vitiligo involving the left side of the trunk and left upper limb with segmental morphea involving the right side of trunk and right upper limb without any deeper involvement is illustrated. There was no history of preceding drug intake, vaccination, trauma, radiation therapy, infection, or hormonal therapy. Family history of stable vitiligo in her brother and a history of type II diabetes mellitus in the father were elicited. Screening for autoimmune diseases and antithyroid antibody was negative. An autoimmune link explaining the co-occurrence has been proposed. Cutaneous mosiacism could explain the presence of both the pathologies in a segmental distribution.

Spindle cell lipoma is a relatively uncommon benign adipocytic tumor that usually presents in subcutaneous fat of adult men. These are a rare form of lipoma, accounting for 1.5% of all lipomatous tumors, with a low rate of local recurrence and no risk of malignant behavior/dedifferentiation. Although few studies addressing the histological findings of spindle cell lipoma have been described, only a few descriptions of fine needle aspiration cytology (FNAC) findings have been documented in literature. We present a case of a 55-year-old male with a nodular swelling over left cheek (in the parotid region), which due to its location as well as prominent myxoid background prompted us to include the lipomatous salivary gland lesions in differential diagnosis. Our objective is to document and delineate the characteristic cytological features of spindle cell lipoma, which may permit a confident diagnosis on FNAC smears.

Gamna gandy bodies are sclerosiderotic granules composed of various amounts of calcium and hemosiderin in hyalinised fibrous tissue. We report a case of an 18 year old girl with solid pseudopapillary tumor of the pancreas in which numerous gamna gandy bodies were present. The pathogenesis of such a finding is unclear. To the best of our knowledge, this is the first report of Gamna Gandy bodies occurring in a solid pesudopapillary tumor of the pancreas.

A case of systemic lupus erythematosus presenting as erythema annulare centrifugum involving atypical sites (face, palm and sole) and raised serum angiotensin-converting enzyme levels has been illustrated.