Diagnostic Dilemma of an Umbilical Mass in a Newborn Infant - a Twin or a Tumor?

BACKGROUND: We present the diagnostic dilemma of a neonate with two umbilical soft tissue masses. Case report: The baby had an umbilical mass herniating through the umbilical cord, and another mass hanging from the umbilical mass by a string of tissue. Both masses were amorphous solid soft tissues and the hanging mass had hair on the surface. Clinical diagnosis was umbilical cord teratoma. However, histopathological examination of the masses showed that tissues representing various organs were arranged in cephalocaudal order as in a fetus, revealing that it was a parasitic twin. The hanging mass was probably the cephalic part and the umbilical mass was malformed torso and limbs. Conclusion: This parasitic omphalopagus heteropagus parasitic twin presented as two amorphous masses without externally identifiable anatomic structure, The parasitic twin of omphalopagus heteropagus may have unusual presentations. Histopathological examination was essential to diagnose whether it is a twin or a tumor.

Prognostic significance of receptor for hyaluronan acid-mediated motility (CD168) in acute pediatric leukemias - assessment of clinical outcome, post induction, end of treatment and minimal residual disease.

INTRODUCTION: The extracellular matrix protein hyaluronan acid plays an active in role in tumor cell proliferation and invasion. Hyaluronan acid receptors, namely CD168 or the receptor for hyaluronan acid-mediated motility (RHAMM) and CD44 have been implicated in promoting malignancy. There is a lacuna in data on the expression of the receptor in pediatric leukemias. METHODS: Pediatric patients with acute leukemia who were diagnosed, treated and followed up in our center were enrolled. The bone marrow biopsies performed prior to treatment were subjected to immunohistochemical staining (54 biopsies: acute lymphoblastic leukemia - 45, acute myeloid leukemia - 9). Blast counts were carried out at diagnosis, end of the induction phase and end of chemotherapy, the minimal residual disease was assessed and follow up details were collected. Positivity was correlated with initial blast count, post-induction blast count, minimal residual disease and patient survival. RESULTS: There was no correlation between the initial blast count and the percentage of blasts with RHAMM expression. The positive correlation between percentage of blasts expressing RHAMM and the post-induction blast count was moderate in acute myeloid leukemia (0.74) and mild in acute lymphoblastic leukemia (0.48). There was a statistically significant difference in RHAMM expression between the two minimal residual disease risk groups (p-value = 0.012) with a negative prognostic effect of RHAMM expression. Moreover, a negative prognostic effect of RHAMM expression was noted when patient survival was considered. CONCLUSION: This study shows that blasts in acute myeloid leukemia show more RHAMM positivity than those of acute lymphoblastic leukemia indicating the aggressive nature of this type of leukemia. In acute leukemias, patients with high percentages of RHAMM-positive blasts had more post-induction blasts, blasts in minimal residual disease and poorer prognosis.

Prognostic significance of receptor for hyaluronan acid-mediated motility (CD168) in acute pediatric leukemias - assessment of clinical outcome, post induction, end of treatment and minimal residual disease

ABSTRACT Introduction: The extracellular matrix protein hyaluronan acid plays an active in role in tumor cell proliferation and invasion. Hyaluronan acid receptors, namely CD168 or the receptor for hyaluronan acid-mediated motility (RHAMM) and CD44 have been implicated in promoting malignancy. There is a lacuna in data on the expression of the receptor in pediatric leukemias. Methods: Pediatric patients with acute leukemia who were diagnosed, treated and followed up in our center were enrolled. The bone marrow biopsies performed prior to treatment were subjected to immunohistochemical staining (54 biopsies: acute lymphoblastic leukemia - 45, acute myeloid leukemia - 9). Blast counts were carried out at diagnosis, end of the induction phase and end of chemotherapy, the minimal residual disease was assessed and follow up details were collected. Positivity was correlated with initial blast count, post-induction blast count, minimal residual disease and patient survival. Results: There was no correlation between the initial blast count and the percentage of blasts with RHAMM expression. The positive correlation between percentage of blasts expressing RHAMM and the post-induction blast count was moderate in acute myeloid leukemia (0.74) and mild in acute lymphoblastic leukemia (0.48). There was a statistically significant difference in RHAMM expression between the two minimal residual disease risk groups (p-value = 0.012) with a negative prognostic effect of RHAMM expression. Moreover, a negative prognostic effect of RHAMM expression was noted when patient survival was considered. Conclusion: This study shows that blasts in acute myeloid leukemia show more RHAMM positivity than those of acute lymphoblastic leukemia indicating the aggressive nature of this type of leukemia. In acute leukemias, patients with high percentages of RHAMM-positive blasts had more post-induction blasts, blasts in minimal residual disease and poorer prognosis.

Neonate with haemophagocytic lymphohistiocytosis secondary to dengue infection: a case report.

We report the first case of haemophagocytic lymphohistiocytosis (HLH) in a neonate secondary to primary Dengue virus infection. This neonate presented in the third week of life with fever, shock and hepatosplenomegaly and was diagnosed to have Dengue infection by serology and HLH was confirmed on bone marrow.

Co-Incidence or Co-Existence? Acute Lymphoblastic Leukaemia in HbE-alpha Thalassaemia: A Case Report with Review of Literature.

Haemoglobin E (HbE) is a Haemoglobin variant that commonly occurs in many places in Asia. As ß thalassaemia and α thalassaemia also occur in the same regions, the co-inheritance of these conditions leads to various phenotypic forms. HbE α thalassaemia is less common and of a milder phenotype than HbE ß thalassaemia. Though malignancies are one of the complications in thalassaemia, occurrence of leukaemia is a rare event. Here we present a case of a two-year-old male child co-presenting with pre B acute lymphoblastic leukaemia (ALL) with MLL rearrangement and HbE alpha thalassaemia. The child is on remission 12 months post-therapy with standard ALL high risk protocol with no minimal residual disease (MRD). Haematological and oncological conditions coexisting at presentation is a challenge to therapy. This case is described for its rarity. Informed consent has been obtained from the parents.

Biological Reference Interval for Hematological Profile of Umbilical Cord Blood: A Study Conducted at A Tertiary Care Centre in South India.

INTRODUCTION: Umbilical cord blood (UCB) a source of hematopoietic stem cells, is also an acceptable sample to assess neonatal sepsis. Though reports are available for stem cell counts very minimal literature is available regarding hematologic parameters, which may vary on ethnicity. AIM: To establish biological reference interval for hematological parameters of umbilical cord blood to guide neonatologists, hematopoietic stem cell transplant specialists and future analysis. MATERIALS AND METHODS: Prospective longitudinal study was done from January 2014 to April 2014 after ethics committee approval. UCB from 120 full term new borns of normal birth weight born out of uneventful pregnancy to mothers aged between 21 to 45 years with hemoglobin above 10g/dL were processed in Beckman Coulter LH780 analyzer for complete blood count and counter checked by peripheral smear. Results tabulated in Microsoft excel are analyzed using IBM SPSS statistics 16 software. RESULTS: Male to female ratio is 1:1.05. There is no difference in the values between males and females. When compared with few studies available, though many values are comparable a few values are not comparable. CONCLUSION: This study can be a useful guide to neonatologists, hematopoietic stem cells transplant hematologists and future analysis.

Pancytopenia - (?) An obstacle in the diagnosis and outcome of pediatric acute lymphoblastic leukemia.

CONTEXT: Acute lymphoblastic leukemia (ALL) ranks first among pediatric malignancies. 8-12% of ALL present with pancytopenia and 2% with hypocellular marrow a. Diagnosis of ALL in the background of pancytopenia and aplastic bone marrow is difficult. AIMS: This study was aimed to compare the clinicopathologic, genetic, and outcome of paediatric ALL patients with and without pancytopenia. SETTINGS AND DESIGN: This is a retrospective cross-sectional study. SUBJECTS AND METHODS: The study included all ALL patients presenting with pancytopenia. The control group included equal number of randomly selected patients with ALL without pancytopenia treated during the same period. Ethics committee approved this study. The demographic, laboratory, and treatment-related details were retrieved from the records and entered in an Excel sheet. STATISTICAL ANALYSIS USED: Data was analyzed with Chi-square test with IBM SPSS statistics 16 software. RESULTS: Diagnosis by peripheral smear is significantly lower (P = 0.015) in comparison with the control group. There is no significant difference in diagnosis between the groups by bone marrow aspirate (P = 0.731) and biopsy (P = 0.849). The diagnosis of leukemia is misdiagnosed as hypo cellular/aplastic marrow in 10% of the pancytopenic patients. Flow cytometry yielded the diagnosis in all the pancytopenic patients. Though cytogenetic abnormalities are more common in pancytopenic group, it is not statistically significant (P = 0.106). There is no significant difference in treatment outcome between the groups (P = 0.0827%). CONCLUSIONS: Clinical expertise is highly essential to evaluate a case of pancytopenia to diagnose leukemia. Pancytopenia is an obstacle in the diagnosis of ALL without immunophenotyping. There is no significant difference in the outcome between the two groups.