We present our data on granulocyte transfusions in children undergoing treatment for cancer and HSCT at our centre and their changing indications. In this retrospective observational analysis of children who received granulocytes from 2007 to 2015, we divided children receiving granulocytes into two groups-the first from January 2007 till December 2013 and the second from January 2014 till December 2015. This division is based on the change in our policy to use granulocytes within 48 h of septicemia as the incidence of drug resistant bacterial strains had increased at our centre. Data on 72 children with febrile neutropenia treated with 230 granulocyte infusions was analyzed. From 2007 to 2013 (n = 48/72), 27/48 (56 %) had culture proven sepsis of which 14 (51 %) were carbapenem resistant gram negative bacilli. 11 of the 27 children survived the crisis (41 %). We then changed our policy to transfuse granulocytes early during sepsis. From 2014 to 2015 (n = 24/72) 22 patients had culture proven sepsis (91 %) of which 20 had carbapenem resistant gram negative bacilli. 12/22 (54 %) with culture proven sepsis survived the episode. The survival rate improved from 41 % in first group to 54 % after early intervention with granulocytes ( value 0.0347). Despite the increased incidence of resistant bacteria during the period of 2014-2015, early use of granulocytes improved survival rate from 41 to 54 %. This intervention cannot be taken in isolation and needs to be offered early in parallel with appropriate antibiotics.

Refractory/relapsed Langerhans cell histiocytosis (LCH) has a difficult course with a guarded prognosis. We used a novel protocol including six cycles of pulse dexamethasone and lenalidomide in four children with LCH refractory to first-line agents and courses of cladribine and cytarabine or single-agent cladribine. All four children completed the protocol without any significant adverse effects and remain in complete and durable remission 15-18 months posttreatment. The novel protocol we propose for relapsed/refractory LCH is cost-effective and outpatient-based with durable remission and minimal toxicity. This is particularly suited for resource-limited settings.

Refractory/relapsed Langerhans cell histiocytosis (LCH) has a difficult course with a guarded prognosis. Salvage treatments available are both expensive and highly toxic. On the basis of the pathophysiology of LCH, we used a novel protocol including pulse dexamethasone and lenalidomide in a child with refractory LCH involving the choroid plexus, which resulted in durable remission with minimal toxicity. The protocol was extrapolated from the FIRST trial for patients with multiple myeloma. We present the clinical course, treatment protocol, and outcome in this child, who is at present disease free and in remission 18 months posttreatment.

Kaposiform hemangioendothelioma is a rare, highly vascular and aggressive soft tissue tumor frequently associated with Kasabach-Merritt phenomenon, usually seen in early infancy. Early diagnosis by means of MRI and tissue biopsy portends a better outcome. Treatment includes surgical excision when feasible and medical management with steroids, propranolol, vincristine and supportive treatment for coagulopathy. We report a 3 months old female infant who was diagnosed, treated successfully and is now in complete remission.Keywords Kaposiform hemangioendothelioma Á Kasabach-Merritt phenomenon Á Vascular soft tissue tumor Á Vincristine Á Steroids Á Remission Three months old, developmentally normal female infant was evaluated for an incidentally noticed ill-defined, nontender, firm and immobile mass in the right paravertebral dorsolumbar region, with features of consumptive coagulopathy. Investigations revealed anaemia, thrombocytopenia and deranged PT/PTT. Ultrasound abdomen (Fig. 2) revealed an ill defined mixed echoeic mass lesion in the retroperitoneum, predominantly in the midline with extension to both paravertebral regions, with few calcific foci, encasing the vessels and infiltrating the pancreas. MRI abdomen (Fig. 1) confirmed the above findings, additionally revealing that the lesion was encasing the abdominal aorta, inferior vena cava and extending to the bilateral renal hila. The lesion was also extending superiorly from the level of the coeliac axis and inferiorly into the mesentery encasing the branches of the superior mesenteric vessels.The child was started on intravenous corticosteroids (methylprednisolone 2 mg/kg/day) and propranolol (0.2 mg/kg/day) to achieve haemostasis. Open biopsy after 7 days of steroids and after consumptive coagulopathy was controlled, confirmed the diagnosis of Kaposiform hemangioendothelioma (KHE). Oral steroids (prednisolone 1 mg/kg) and propranolol were continued for 12 weeks