Ho/01(P) incidence of iron deficiency in non anaemic children

HO/05(P) BRONCHOALVEOLAR LAVAGE CYTOLOGY IN 15 THALASSEMIA MAJOR CHILDREN

Ankit Parakh, A.P.Dubey, G. R. Sethi, .Shyma Jain     

Department of Paediatrics And Pathology Maulana Azad Medical College, New Delhi-110002

Introduction: Despite greater than 20 years of study, the cause of pulmonary dysfunction in thalassemia is not established. Data is still inconclusive whether the cause of pulmonary abnormalities is hemosiderosis.Aims And Objectives: To study the cause of pulmonary dysfunction by bronchoalveolar lavage (BAL) cytology. Material and methods: 31 thalassemics were recruited. All underwent pulmonary function tests (spirometry, lung volumes and single breath carbon monoxide diffusion capacity). Serum ferritin was measured. Those with abnormal pulmonary function tests or impaired diffusion capacity or both (15 of 31) were further subjected to bronchoalveolar lavage. Total cell count was measured. Differential cell count was done on Giemsa and PAP stained smears. Iron laden macrophages were identified on Perl's stain (Prussian blue reaction). Results: Iron laden macrophages were demonstrated in majority of the broncho alveolar lavage (14 out of 15 patients). The total count was normal in all the patients. Differential count did not reveal any specific abnormality. Lymphocytic alveolitis was demonstrated in 2 patients. In one of these patients neutrophils and eosinophils were also more than the upper limit of normal. Conclusions: The presence of Iron laden macrophages in bronchoalveolar lavage in majority of cases corroborates the hypothesis of an iron induced toxic mechanism, although a confirmation can be given by a lung biopsy. Lymphocytic alveolitis was demonstrated in 2 patients. It might indicate a hypersensitivity or allergic reaction (allergic alveolitis) to iron, which is a heavy metal. This issue needs further studies with a larger sample size.
HO/06(O) NEUROLOGICAL COMPLICATIONS IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKAEMIA

Leni G Mathew, Anila Chacko, Jincy Jeba, Santhosh K, Vanathi Sethupathy, Julius Scott

Paediatric Oncolgy, Department of Child Health, CMC, Vellore.
Aim: To study the neurological complications in children receiving treatment for Acute Lymphoblasic Leukaemia.(ALL) Patients and Methods: Children with ALL attending paediatric Oncology unit of CMC, Vellore from Aug 2003 till Aug 2005 were included and their clinical records were reviewed. Results: 110 children received treatment for ALL. 66% had preB ALL, 20% T ALL, 5% biphenotypic ALL and the remaining were unclassified. 23 children had various neurological complications. 4 had paraparesis at diagnosis, 8 children developed various neurological problems following vincristine; 5 had peripheral neuropathy and 3 had ptosis. Complications of Intrathecal methotrxate included opisthotonic posturing in 4 and leucoencehalopathy in 2. A child with T ALL developed intractable seizure and hyponatremia. 2 children had ADEM, 1 had CNS toxoplasmosis and another child had traumatic injury following a fall. Conclusion: Incidence of neurological complication in children with ALL in our centre was 20%. Majority were related to the treatment and it varied in severity from mild and transient to chronic or fatal.
HO/07(O) COMPARATIVE STUDY OF DESFERRIOXAMINE AND DEFERIPRONE (L-1) IN -THALASSEMIA MAJOR

B. M. John, P. L. Prasad,

Graded Specialist (Pediatrics), 7 Air Force Hospital, Kanpur Cantt. -208004
Background: The treatment of thalassemia major revolves around effective blood transfusion and prevention of iron overload. Therapy with the time tested parenteral iron chelator desferrioxamine (DFO) is fraught with difficulty with respect to availability and compliance. The last two decades have witnessed use of an oral iron chelator deferiprone (DFP) with variable results. Objectives: (i) To compare the efficacy of desferrioxamine and deferiprone in lowering body iron using 24 hour urinary iron excretion and serum ferritin. (ii) To look at the adverse effects of therapy. Methods: A randomised prospective study was done with 40 thalassemic children between 3 to 11 years over a 20 month period. 20 children were recruited in each treatment group with DFO group receiving s/c infusion of desferrioxamine 25-50mg/kg/day, 5 times a week and DFP group receiving deferiprone 75mg/kg/day. 24hour urinary iron excretion and serum ferritin levels were studied before and after use of DFO and DFP and compared. Adverse effects were also studied simultaneously. Results: There was a significant rise in mean 24-hour urinary iron excretion from 0.88mg/day to 14.73mg/day in DFO group and from 0.87mg/day to 11.5mg/day in the DFP group (p<0.05 in both groups –paired t test). There was also a significant fall in mean serum ferritin in DFO group from 4683.20ng/ml to 1983.85ng/ml and in the DFP group from 4451.29ng/ml to 2107.24ng/ml (p<0.05 in both groups –paired t test). There was no significant difference in the percentage change in iron excretion or drop in ferritin levels when the two groups were compared with each other (p>0.05 by unpaired t test). The adverse effects in DFO group were minor pain/swelling in 15% cases. Adverse effects in DFP group included GI disturbances in 10% cases, arthralgias in 29% and arthritis in 20%. None necessitated discontinuation of chelators. Conclusion: Deferiprone may be as effective as desferrioxamine in the treatment of thalassemia major with only few mild side effects.

HO/08(P) FANCONI’S ANAEMIA WITH ACUTE MYELOID LEUKAEMIA (M7) IN A CHILD

Nalini K Pati, Peter J Shaw,

BMT Unit, Department of Oncology, The Royal Alexandra Hospital for Children, Westmead, Sydney, Australia

Fanconi’s anaemia (FA) is a well know chromosomal instability disorder with a high risk of developing acute myeloid leukaemia (AML) and certain solid tumours. The development of AML (M7) in children with FA is extremely rare. We describe a 14 year old boy with FA who presented to the hospital with an unexpected high platelet count. The patient was diagnosed with FA at birth with skeletal dysmorphism, cardiac and kidney anomalies. Chromosomal breakage studies confirmed the diagnosis. He maintained low blood counts, never required transfusion and a suitable matched unrelated donor was identified, with a recommendation to proceed to BMT if he became transfusion dependent. His family moved and he was lost to follow up for 5 years. He represented with a platelet count of 995 x 10⁹/L. Bone marrow evaluation revealed a blast count of 60% with abnormal megakaryocytes. Flow Cytometry revealed positivity for CD13 (73%), CD33 (73%), CD41 (69%), CD34 (73%) and CD7 (86%), consistent with AML (M7). Cytogenetics including FISH was normal and PCR testing was negative for the translocations t(15;17), t(8;21) and inv16. The literature has many examples of patients with FA and malignancy, but minimal advice on the treatment of AML in these patients. In the absence of specific information and given the strong positivity for CD33, therapy was initiated with Gemtuzumab 5mg (3mg/m²) 14 days apart, whilst plans were made to reactivate the donor and proceed to urgent BMT. After 2 doses of Gemtuzumab his count fell to a Hb of 105g/L, White count 0.8 x 10⁹/L and platelets of 122 x10⁹/L. A repeat bone marrow evaluation revealed a megakaryoblasts of 20%. He had no toxicity related to the therapy and, after some interim mild chemotherapy, proceeded to BMT. Gemtuzamab provides targeted therapy for AML with none of the usual side-effects of chemotherapy, making it suitable for use in the setting of FA. After years of thrombocytopenia, a rising platelet count should prompt an early marrow evaluation to look for myelodysplasia or AML