CLINICAL PROGRESSION INTRODUCTION METHOD CONCLUSION REFERENCES Philadelphia Positive Chronic Myelogenous Leukemia in Pediatric Patients T Mukhopadhyay, R Awale, M Setia, R Maji , G Chhabra S Sharma, AK Mukhopadhyay Department of Laboratory Medicine All India Institute of Medical Sciences, New Delhi INTRODUCTION CLINICAL PROGRESSION Chronic Myelogenous Leukemia (CML) results from somatic mutation in hemopoeitic stem cells leading to clonal expansion and proliferation of granulocytic series. Incidence of CML in children is very rare, accounting for less than 3% of leukemia cases in children and adolescents. [1] Annual incidence: 1 per million in western world.[1] In India, age specific incidence rate of 0.04 per 100,000 was reported during 2001-2005. [2] Pediatric CML accounts for 10-15% of all CML, clinical presentation being similar to that of adults. Both cases were treated and put on follow up. Case 1 lost to follow up and presented after two years in blast crisis. Whereas case 2 improved. Clinical details, examination findings and laboratory investigations at follow up are tabulated in Table 2. TABLE 2: Case details Case 1 Case 2 Age/ Sex 8years / Female 15years/ Male Complaints of Low grade fever, increasing pallor X 2 days Cough X 3days left sided mass per abdomen increasing rapidly in size Asymptomatic On examination Pallor, periorbital puffiness Massive splenomegaly No pallor, lymphadenopathy or organomegaly Hemoglobin (gm %) 6.3 13.7 Total Leukocyte count (cells/cumm) 2,04,000 7,200 Differential Leukocyte count (%) Blast 28 - Promyelocyte Myelocyte 22 Metamyelocytes 3 Neutrophil 36 80 Lymphocytes 4 15 Monocyte 2 Eosinophil Basophils 1 NRBC (cells/100 WBC) Platelet count (cells/cumm) 2,14,000 1,30,000 Flowcytometry analysis cMPO+ , CD 34+, CD 134+, CD33+ , CD117+ , CD 45+ cCD 79a- , cCD3- , CD 19- cMPO+ , CD34+ , CD 45+ METHOD Patients presented to pediatric OPD in AIIMS with complaints of generalised weakness and mass in left hypochondrium. Complete blood count revealed leucocytosis. Diagnosis for CML was confirmed on the basis of morphology, cytochemistry, and molecular testing for the expression of BCR-ABL transcript fusion p210 by RT/PCR. Clinical details at presentation are tabulated in Table 1. SM Figure 1: Philadelphia chromosome  translocation t(9;22) occuring in CML. Figure 2: Per abdomen examination showing palpable enlarged spleen(SM). TABLE 1: Case details Case 1 Case 2 Age/ Sex 6years / Female 14years/ Male Complaints of Low grade fever, increasing pallor, left sided mass per abdomen X 4 months Low grade fever, left sided lump in abdomen X 2 months On examination Pallor Inguinal, axillary lymphadenopathy hepatosplenomegaly Splenomegaly Hemoglobin(gm %) 8.3 14.9 Total Leukocyte count (cells/cumm) 1,08,000 70,700 Differential Leukocyte count (%) Blast 1 Promyelocyte 4 - Myelocyte 15 Metamyelocytes 20 2 Neutrophil 39 80 Lymphocytes 6 8 Monocyte Eosinophil Basophils 5 3 NRBC (cells/100 WBC) Platelet count (cells/cumm) 2,60,000 2,68,000 Philadelphia chromosome (RT-PCR) POSITIVE Figure 5: Peripheral smear (400X) of CML in blast crisis in case 1. Figure 6: Peripheral smear (1000X) of case 1 showing Blast, Myelocyte, metamyelocyte. CONCLUSION One must have high sense of suspicion when leucocytosis is found even in pediatric age, as CML could be one of the differential diagnosis of leucocytosis. When differential count is also suggestive, should be followed up by molecular genetic analysis for presence of philadelphia chromosome in conjuction with bone marrow morphology and immunophenotyping. REFERENCES Rels et al. Cancer Incidence and Survival Among Children and Adolescents: United States SEER Program, 1975-1995. NIH pub no. 99-4649. Bethesda, MD: National Cancer Institute;1999. Au et al. Chronic Myeloid Leukemia in Aisa, Int J Hematol (2009) 89:14-23. WHO (2008) WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. International Agency for Research on Cancer: Lyon Figure 3: Leishman stained peripheral smear (400X) of case 1 showing showing marked leucocytosis and shift to left. Inset: MPO positivity on cytochemistry. Figure 4: Leishman stained peripheral smear (1000X) of case 2 showing features of CML in chronic phase.