Pediatric chronic myeloid leukemia in myeloid blast crisis

Chronic myeloid leukemia (CML) accounts for 2-3% of childhood leukemias. About 5% of cases present in a blastic phase of CML which clinically and morphologically mimics more common acute leukemias of childhood. We report a case of a 3-yearold male who presented with gradual onset swelling of the abdomen and extremities along with generalized weakness. Examination revealed massive splenomegaly, pallor, and pedal edema. Initial workup showed anemia, thrombocytopenia, and leukocytosis (120,000/uL) with a blast percentage of 35%. Blasts were positive for CD13, CD33, CD117, CD34 and HLA-DR, and stained negative for Myeloperoxidase and Periodic Acid Schiff. Fluorescence in situ hybridization was positive for b3a2/e14a2 junction BCR-ABL1 transcript and negative for RUNX1-RUNX1T1/t(8;21), clinching the diagnosis of CML in myeloid blast crisis. The patient expired within 17 days of diagnosis and initiation of therapy

Pediatric chronic myeloid leukemia in myeloid blast crisis

Chronic myeloid leukemia (CML) accounts for 2-3% of childhood leukemias. About 5% of cases present in a blastic phase of CML which clinically and morphologically mimics more common acute leukemias of childhood. We report a case of a 3-yearold male who presented with gradual onset swelling of the abdomen and extremities along with generalized weakness. Examination revealed massive splenomegaly, pallor, and pedal edema. Initial workup showed anemia, thrombocytopenia, and leukocytosis (120,000/uL) with a blast percentage of 35%. Blasts were positive for CD13, CD33, CD117, CD34 and HLA-DR, and stained negative for Myeloperoxidase and Periodic Acid Schiff. Fluorescence in situ hybridization was positive for b3a2/e14a2 junction BCR-ABL1 transcript and negative for RUNX1-RUNX1T1/t(8;21), clinching the diagnosis of CML in myeloid blast crisis. The patient expired within 17 days of diagnosis and initiation of therapy

Successful management of total placenta previa with placenta accreta presenting with massive obstetric hemorrhage: A case report

Placenta previa and placenta accreta are important causes of serious fetal and maternal morbidity and even mortality necessitating hysterectomy. We report a case of total placenta previa with accreta in a 39-year-old female in the 32+2 weeks of pregnancy with successful delivery by cesarean. However, hysterectomy was performed to control postpartum hemorrhage

Henoch-Schonlein Purpura in Northeast India: Peculiarities in Presentation

Background and Aim: Henoch-Schönlein purpura (HSP) is the most common childhood vasculitis characterized by leukocytoclastic vasculitis. This study was done to describe the presentation and immediate outcome of children admitted to HSP at our Institute. Methods: This retrospective study was conducted on children with HSP admitted to our department over a period of 7 years (January 2010 until December 2016). Results: Twenty-three children with a diagnosis of HSP were identified during the study period. The mean age was 9.4 years (4 years to 16 years). There were 15 girls and 8 boys with a male: female ratio of 1:1.9. The youngest child was 4 years old and most of the children (73.9%) were in the age group 5-12 years. Forty percent of the children presented between January and March. Major manifestations were rash (100%), joint pain (52%), renal involvement (52%), and abdominal pain (47.8%). Three (13.0%) children presented with systemic manifestations before the appearance of the rash. One child had MPGN 2 years before the onset of rash. There was no mortality. Most of the children recovered well; six (26%) had persistent hypertension and three (13%) had persistent proteinuria. Hypertensive emergency was seen in two children. One child had intussusception that resolved spontaneously. Conclusion: This study is the first study of Henoch Schonlein purpura from northeast India documenting certain peculiarities in the presentation. The results indicate a wide spectrum of presentations in HSP

An Audit of primary ovarian pregnancy in a tertiary care hospital

Background: Ovarian pregnancy is uncommon form of ectopic pregnancy with increasing trend in its incidence. Various risk factors have been implicated like intrauterine contraceptive devices, fertility treatments, pelvic inflammatory disease (PID), and endometriosis.Methods: This is a retrospective observational study done over a period of 10 years from January 2008 to December 2017.All the patients, who were diagnosed as primary ovarian pregnancy based on Spielberg’s criteria, were included in the study.Results: There were 6 cases (6.3%) of primary ovarian pregnancy out of the 95 cases of ectopic pregnancies.  The age of the patients ranged from 28 years to 43 years with a mean age of 31.8 years. Amenorrhea and acute pain abdomen was present in all the patients. Laparotomy was done in all the cases. Excision of the sac with partial ovariectomy was done in 2 cases (33.3%).Oophorectomy with salpingectomy was done in the rest of the 4 cases (66.7%). Histopathological confirmation was done in all the cases.Conclusions: Ovarian pregnancy is uncommon form of ectopic pregnancy with increasing trend in its incidence. Management of choice is laparoscopy. Laparotomy is done if there are signs of rupture

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised

Demographic pattern and histopathological profile of neuroendocrine neoplasms diagnosed at a tertiary care center in North East India

Background: Neuroendocrine tumors (NENs) are a group heterogenous group of tumors that can arise in any organ in the body and have a wide range of aggressiveness. Aims and Objective: To compare the frequency of NENs in our setup to those reported in the literature to age, site, and degree of differentiation by doing a retrospective study. Materials and Methods: Cases of NENs that were diagnosed in the Department of Pathology, in our hospital over the past 5 years were studied considering the age, location, and degree of differentiation. A fresh panel of immunohistochemistry (IHC) was conducted for those cases where IHC was not done. Results: A total of 46 cases of NENs were reviewed. About 56.5% (26 cases) were males and 43.5% (20 cases) were females, with a median age of 46 years. In our study, most of the tumors are found in the gastro-entero-pancreatico-hepatobilliary group followed by the NENs of the endocrine gland (21.7%) and broncho-pulmonary group (15.2%). NENs were graded based on mitotic count and/or Ki-67 labeling index, and/or the presence of necrosis. In total 21 cases (45.7%) had G1 grading, 7 cases (15.2%) had G2 grading, 2 cases (4.3%) had a G3 grading, and 16 cases (34.7%) were graded as neuroendocrine carcinoma. Conclusion: As the majority of the studies do not include benign NENs and those arising from the endocrine glands, therefore comparison of our results can be difficult. This is the first attempt to study the NENs from North East India and to analyze their clinicopathological features

Modulation of inhibitory activity of xylanase - α-amylase inhibitor protein (XAIP): binding studies and crystal structure determination of XAIP- II from Scadoxus multiflorus at 1.2 Å resolution

Background: Plants produce a wide range of proteinaceous inhibitors to protect themselves against hydrolytic enzymes. Recently a novel protein XAIP belonging to a new sub-family (GH18C) was reported to inhibit two structurally unrelated enzymes xylanase GH11 and α -amylase GH13. It was shown to inhibit xylanase GH11 with greater potency than that of α-amylase GH13. A new form of XAIP (XAIP-II) that inhibits α-amylase GH13 with a greater potency than that of XAIP and xylanase GH11 with a lower potency than that of XAIP, has been identified in the extracts of underground bulbs of Scadoxus multiflorus. This kind of occurrence of isoforms of inhibitor proteins is a rare observation and offers new opportunities for understanding the principles of protein engineering by nature. Results: In order to determine the structural basis of the enhanced potency of XAIP-II against α-amylase GH13 and its reduced potency against xylanase GH11 as compared to that of XAIP, we have purified XAIP-II to homogeneity and obtained its complete amino acid sequence using cloning procedure. It has been crystallized with 0.1 M ammonium sulphate as the precipitating agent and the three-dimensional structure has been determined at 1.2 Å resolution. The binding studies of XAIP-II with xylanase GH11 and α-amylase GH13 have been carried out with surface plasmon resonance (SPR). Conclusion: The structure determination revealed that XAIP-II adopts the well known TIM barrel fold. The xylanase GH11 binding site in XAIP-II is formed mainly with loop α3-β3 (residues, 102 - 118) which has acquired a stereochemically less favorable conformation for binding to xylanase GH11 because of the addition of an extra residue, Ala105 and due to replacements of two important residues, His106 and Asn109 by Thr107 and Ser110. On the other hand, the α-amylase binding site, which consists of α-helices α6 (residues, 193 - 206), α7 (residues, 230 - 243) and loop β6-α6 (residues, 180 - 192) adopts a stereochemically more favorable conformation due to replacements of residues, Ser190, Gly191 and Glu194 by Ala191, Ser192 and Ser195 respectively in α-helix α6, Glu231 and His236 by Thr232 and Ser237 respectively in α-helix α7. As a result, XAIP-II binds to xylanase GH11 less favorably while it interacts more strongly with α-amylase GH13 as compared to XAIP. These observations correlate well with the values of 4.2 × 10-6 M and 3.4 × 10-8 M for the dissociation constants of XAIP-II with xylanase GH11 and α-amylase GH13 respectively and those of 4.5 × 10-7 M and 3.6 × 10-6 M of XAIP with xylanase GH11 and α-amylase GH13 respectively