Case report:
An 11-year-old boy, diagnosed to have XP at the age of 6 months, was
referred to us with history of fever for 10 days, vomiting and decreased
appetite for 5 days. There was no history of bone pains/ bleeding
manifestations. Initially he was treated with ayurvedic medications and
due to non-improvement, a blood test was done which revealed severe
anaemia and thrombocytopenia. Child received packed red cell and
platelet transfusions at a nearby tertiary care centre, underwent bone
marrow studies, and was referred to our centre with a diagnosis of acute
leukaemia for further management.
On physical examination, he had multiple diffuse hyperpigmented macules
all over the body with sparing of palms and soles (Figure 1). There was
conjunctival congestion with bilateral pterygium (Figure 1) and mild
photophobia. There was no pallor, icterus, cyanosis, clubbing,
lymphadenopathy or bleeding manifestations. Liver was not enlarged,
spleen was palpable 2 cm below right costal margin and testes were
normal. The rest of systemic examination was normal.
Blood investigations revealed Haemoglobin: 9.9 gm%, Total leucocyte
count: 1100/mm3, Platelets:
14,000/mm3. Differential Count: neutrophils 31.3%,
Lymphocytes: 66.8%, Eosinophils: 0.2%, Monocytes: 1.2%, Basophils
0.5%. Uric acid: 3.8 mg/dl. Renal and liver function tests were normal.
There was no biochemical evidence of tumour lysis syndrome.
Peripheral blood smear showed pancytopenia with 6% immature
cells/blasts. Chest X ray was normal. Bone marrow aspiration showed 60%
blasts, which were myeloperoxidase negative (Figure 2) and bone marrow
flow cytometry was suggestive of B- Lymphoblastic Leukaemia (CD 5+, CD
10+, CD 19+, CD 20+, cyCD79a+, CD 33 dim positive, CD 34 -, HLA DR +).
Cerebro spinal fluid (CSF) study was normal.
Child was treated using Modified BFM (Berlin-Frankfurt-Münster) ALL
Protocol. Induction phase included prednisolone, vincristine,
daunorubicin, L- Asparaginase and intrathecal methotrexate. He tolerated
induction chemotherapy fairly well. He had good prednisolone response on
day 8 of steroids with no blasts in the peripheral blood. He had
hyperbilirubinemia and transaminitis after day 15 vincristine [S.
Bilirubin:1.9 mg/dl (grade 2 CTCAE), SGPT: 248 U/L (grade 2 CTCAE),
SGOT: 105 U/L (Grade 1 CTCAE)] during induction chemotherapy, which
had improved spontaneously. Hence subsequent Day 22 and Day 29
vincristine were administered at 50 % dose, which he tolerated well
without development of further hyperbilirubinemia and transaminitis.
Post induction chemotherapy, his bone marrow was in remission.
Subsequently, child completed Phase 1B with Cyclophosphamide, 6-
Mercaptopurine and Cytosine Arabinoside along with intrathecal
methotrexate without any complications. Child subsequently completed
interim maintenance with 6- Mercaptopurine and Methotrexate without any
toxicities. Child then received protocol M with high dose methotrexate
(3 grams/m2 for cycles 1 and 2 and 5
gram/m2 for 3 and 4 cycles). He developed grade 2
mucositis after cycle 2. He also developed blood stream infection with
Pseudomonas aeruginosa during cycle 3 of protocol M and was treated
successfully with Cefoperazone- Sulbactam. Child completed
re-intensification phase uneventfully. He is now on maintenance
chemotherapy. After six cycles of maintenance, he developed
hyperbilirubinemia (S. Bilirubin 2 mg/dL, Grade 2 CTCAE), for which
Vincristine dose was reduced by 50%. He did not have any episodes of
neutropenia during maintenance.