Objective To study the profile of children with Hemophagocytic Lymphohistiocytosis (HLH) in a tertiary care hospital for children. Methods A retrospective analysis of case records of 52 children diagnossed with HLH was performed. Results Of the 52 children 13% (n = 7) had Familial HLH and 87% (n = 45) had secondary HLH (sHLH). Common manifestations were fever (100%), organomegaly (87%), respiratory distress (54%), neurological symptoms (31%) and skin rashes (26.2%). Anemia and thrombocytopenia were present in 51% and 73% respectively. Hyperferritinemia was present in 96% and hypofibrinogenemia in 42% and high lactate dehydrogenase (LDH) in 91%. Bone marrow examination showed hemophagocytosis in 80%. Most common etiology among infections was viral infections (67%), of which Dengue was the most common (52%). Among children with sHLH 51% received supportive care only. Thirty-seven percent (n = 17) received intravenous (IV) immunoglobulin and steroids. Of these 77% (n = 35) recovered completely. Children with familial HLH were initiated on HLH 2004 protocol but all of them expired due to disease progression. Conclusions Identifying HLH early and managing it, poses a significant challenge. Prompt recognition and initiation of immunosuppressive therapy is extremely important for the better outcome; hence high clinical suspicion and structured work up including immunological, and genetic studies is required. It may be difficult to differentiate primary and secondary HLH in many instances unless genetic analysis is done. Identification of familial HLH is necessary for early referral to Hematopoietic Stem Cell Transplantation (HSCT). Hence screening for primary HLH needs to be considered in all children with HLH.

Aims The aim was to study cytogenetics and molecular genetic profile in pediatric B-acute lymphoblastic leukemia (ALL) and correlate it with induction outcomes. Subjects and Methods A retrospective study of cytogenetics and molecular genetics of 98 children with B-cell ALL from January 2013 to May 2018 was done. Cytogenetics and molecular genetics were done in the bone marrow using multiplex reverse transcription polymerase chain reaction and G-banded karyotyping, respectively. Minimal residual disease (MRD) assessment was done at the end of induction by flowcytometry. Results Of the 98 children, 83 (84.6%) had evaluable cytogenetics, with 11 (13.25%) being abnormal karyotypes. Of the 11 abnormal karyotypes, seven children (8.4%) had hyperdiploidy, one had hypodiploidy, and three had miscellaneous findings. In molecular genetics, TEL-AML1 (ETV6/RUNX1)[t(12;21)] was the most common fusion gene abnormality (12.2% [12/98]), followed by E2A-PBX1 [t(1;19)] (5%), BCR/ABL1 [t(9;22)] (3%), and MLL-AF4 [t(4;11)] (1%). All the 98 children attained morphologic remission at the end of induction. All children with hyperdiploidy (7/7) attained remission and MRD negativity, but one expired during maintenance chemotherapy of disseminated tuberculosis. The child with hypodiploidy was MRD-positive. Three (25%) children with t (12;21) were MRD-positive. All children with Ph + ALL, t(1:19), and t(4;11) were MRD-negative. Fifty-two children had no detected abnormalities, six of whom had MRD positivity (11.5%). Conclusion Cytogenetic and molecular genetic subgrouping prognosticates ALL outcomes. Although 25% of TEL-AML + children had MRD positivity, larger studies are required to validate the same. End-of-induction MRD outcomes did not correlate with chromosomal aberrations.

Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis which occurs either idiopathically or associated with various systemic diseases and malignancies. Although the association of PG with myeloid malignancies is well known, its association with lymphoid malignancy especially in children is extremely rare. We describe PG in association with acute lymphoblastic leukemia in a 4-year-old child, an occurrence which has been reported just once previously to the best of our knowledge.

Pediatric Blood & CancerVolume 66, Issue 4 e27574 LETTER TO THE EDITOR A novel mutation in the MECOM gene causing radioulnar synostosis with amegakaryocytic thrombocytopenia (RUSAT-2) in an infant Ajeitha Loganathan, Corresponding Author drajeitha@gmail.com orcid.org/0000-0001-8525-1252 Department of Pediatric Hematology and Oncology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India Correspondence Ajeitha Loganathan, Department of Pediatric Hematology Oncology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai 600 034, Tamil Nadu, India. Email: drajeitha@gmail.comSearch for more papers by this authorDeenadayalan Munirathnam, orcid.org/0000-0002-1316-0746 Department of Pediatric Hematology and Oncology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, IndiaSearch for more papers by this authorThangadorai Ravikumar, Department of Pediatrics, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, IndiaSearch for more papers by this author Ajeitha Loganathan, Corresponding Author drajeitha@gmail.com orcid.org/0000-0001-8525-1252 Department of Pediatric Hematology and Oncology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India Correspondence Ajeitha Loganathan, Department of Pediatric Hematology Oncology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai 600 034, Tamil Nadu, India. Email: drajeitha@gmail.comSearch for more papers by this authorDeenadayalan Munirathnam, orcid.org/0000-0002-1316-0746 Department of Pediatric Hematology and Oncology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, IndiaSearch for more papers by this authorThangadorai Ravikumar, Department of Pediatrics, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, IndiaSearch for more papers by this author First published: 10 December 2018 https://doi.org/10.1002/pbc.27574Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume66, Issue4April 2019e27574 RelatedInformation