Narsoplimab for severe transplant-associated thrombotic microangiopathy.

BACKGROUND: Transplantation-associated thrombotic microangiopathy (TA-TMA) is an endothelial injury syndrome linked to the overactivation of complement pathways. It manifests with microangiopathic hemolytic anemia, consumptive thrombocytopenia, and microvascular thrombosis leading to ischemic tissue injury. Mannose residues on fungi and viruses activate the mannose-binding lectin complement pathway, and hence activation of the lectin pathway could be one of the reasons for triggering TA-TMA. Narsoplimab, a human monoclonal antibody targeting MASP-2 is a potent inhibitor of the lectin pathway. We describe the transplant course of a pediatric patient who developed TA-TMA following Candida-triggered macrophage activation syndrome and was treated with Narsoplimab. The data collection was performed prospectively. CASE PRESENTATION: The six-year-old girl underwent a human leucocyte antigen (HLA) haploidentical hematopoietic stem cell transplant using post-transplant Cyclophosphamide for severe aplastic anemia. In the second week of the transplant, the patient developed macrophage activation syndrome necessitating treatment with steroids and intravenous immunoglobulin. Subsequently, USG abdomen and blood fungal PCR revealed the diagnosis of hepatosplenic candidiasis. Candida-triggered macrophage activation syndrome responded to antifungals, steroids, intravenous immunoglobulin, and alemtuzumab. However, the subsequent clinical course was complicated by thrombotic microangiopathy. The patient developed hypertension in the 2nd week, followed by high lactate dehydrogenase (1010 U/L), schistocytes (5 per hpf), low haptoglobin (< 5 mg/dl), thrombocytopenia, and anemia in the 3rd week. Ciclosporin was stopped, and the patient was treated with 10 days of defibrotide without response. The course was further complicated by the involvement of the gastrointestinal tract and kidneys. She had per rectal bleeding with frequent but low-volume stools, severe abdominal pain, and hypoalbuminemia with a rising urine protein:creatinine ratio. Narsoplimab was started in the 5th week of the transplant. A fall in lactate dehydrogenase was observed after starting Narsoplimab. This was followed by the resolution of gastrointestinal symptoms, proteinuria, and recovery of cytopenia. The second episode of TA-TMA occurred with parvoviraemia and was also successfully treated with Narsoplimab. CONCLUSION: Lectin pathway inhibition could be useful in treating the fatal complication of transplant-associated thrombotic microangiopathy.

PICC fracture and embolization into pulmonary artery - a rare but potentially life-threatening complication: A case report.

Peripherally inserted central catheter (PICCs) are popular means of long-term intravenous access in oncology patients. Fracture and embolization are rare but potentially serious complications. Here we present an unusual fracture of the PICC line in a 9-year-old boy with Ewing's sarcoma with embolization to the right ventricle (RV) and right pulmonary artery (RPA) which was retrieved percutaneously by trans-catheter snare assisted retrieval. Adequate care and precautions like handling by trained nursing staff/parental education must be undertaken to prevent such complications.

X-Linked Hyper IgM Syndrome Presenting with Recurrent Tuberculosis-a Case Report.

The hyper IgM syndromes are a group of rare primary immunodeficiency disorders. Currently 6 classes of HIGM are described. X-linked HIGM is also called the type 1 HIGM is the commonest variant in which children present in early infancy with features of combined immunodeficiency. Tuberculosis is a very rare presentation as a presenting symptom in HIGM. Here, we describe a child with XHIGM with recurrent tuberculosis.

The role of lymph node sampling and radiotherapy in the management of the axilla in early breast cancer.

Management of the axilla in early breast cancer is an issue of ongoing debate. We reviewed our experience in 312 patients who underwent axillary lymph node sampling between 1994 and 1998, of whom 81 patients (24%) had axillary lymph node metastasis. There have been two axillary recurrences, one associated with local recurrence to the breast and one presenting with distant metastasis. There were no patients with isolated axillary disease as their only site of recurrence and no axillary failures in the node-positive group treated with axillary sampling and radiotherapy. Axillary lymph node sampling effectively stages the axilla. This can safely be followed by radiotherapy to the axilla in case of lymph node metastasis. Axillary lymph node sampling forms a sound basis to develop new techniques, such as sentinel lymph node biopsy currently investigated by ongoing trials.