CASE REPORT;
A 9 years old child presented to oncology clinic in September 2020 with six months history of fever, fatigue and gradually increasing bilateral neck swellings. There was no complain of cough, respiratory distress, bone pains, peteciae or bruises. He was pale looking, active and alert child having weight of 22 Kg. BCG scar was present. He had generalized lymphadenopathy, largest were bilateral cervical lymph nodes 5 by 5 cm, discrete, firm, non-tender with normal overlying skin. He had hepatomegaly of 4 cm below right costal margin with splenomegaly of 12 cm below left costal margin. Rest of systemic examination was normal. His Haemoglobin was 6.9 gm/dl, WBC 168.4 8*103/l, neutrophils 36%, lymphocytes 02%, eosinophils 07%, monocytes 02%, promyelocytes 02%, myelocytes 26%, metamyelocytes 16%, basophils 07%, blasts 02%, MCV 80.1 fl, MCH 26.4 pg, Peripheral blood film shows macrocytosis, anisocytosis, and poikilocytosis. Nucleated red blood cells 03/100 WBC. No hemoparasites were identified. Platelets 72,000/l. serum uric acid 7.2 md/dl, potassium 4 mmol/l, LDH 780. Echocardiography, hepatitis B & C screening, calcium, phosphate, liver and renal function tests were normal. CT neck, chest, abdomen showed bilateral cervical, axillary, supraclavicular, mediastinal and abdominal lymphadenopathy, hepatosplenomegaly and splenic parenchymal defects suggesting lymphoproliferative disorder. Flow cytometry on bone marrow aspirate was inconclusive showing less than 5 % blasts. Bone Marrow aspirate morphology showed Hyper cellular fragments showing active trilineage haematopoiesis. Blasts constitute 5%of nucleated marrow cells. Plasma cells constitute 3% of nucleated marrow cells. No hempparasites identified. Blasts cell constitute 08% of nucleated marrow cells. BM differential was myelocytes 40%, metamyelocytes 02%, erythroids 05%, neutrophils 45%, and blasts cells 08%. Bone Marrow trephine biopsy was adequate length with normal bony trabeculae, reduced fat spaces, and 95% cellularity (Fig1). Erythropieiss supressed with hyperplastic myelopoiesis, megakaryopoiesis seen. Peripheral film and bone marrow findings were suggestive of Chronic Myeloproliferative Disorder most likely Chronic Myelogenous Leukaemia in chronic Phase. Excisional biopsy of cervical lymph node was CD 3 positive, CD 20 negative, Tdt positive, cyclin D-1 negative, and KI 67 High (>80%) consistent with T cell Lymphoblastic Lymphoma /Leukaemia (Fig11). BCR-ABL Translocation was not detected on RT-PCR. Differential diagnosis considered included CML with Lymphoma, lymphadenopathy as an extrahematopietic manifestation of CML or myeloid sarcoma. Considering the rarity of association of T lymphoblastic Lymphoma/leukaemia with BCR ABL negative CML in paediatric age group, both the cervical lymph node biopsy and Bone marrow aspiration biopsy were sent for external review, after discussion in tumour board meeting. Both reports corresponded to and confirmed the primary diagnosis of T lymphoblastic Lymphoma with BCR ABL negative CML which in unheard of, in paediatric population. The family was appropriately counselled. The child was started T lysis and pneumocystis jerovecci prophylaxis, hyperhydraion, and antibiotics. Literature was searched to make optimal treatment plan for this child. There was paucity of data to make exact recommendation of treatment. Family was counselled about bone Marrow Transplant when his lymphoblastic is in remission, to which they did not consent. The child was started on UKALL 2011 induction.