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Beryl Thyer
Memorial Africa Trust
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Burkitt's Lymphoma: 2 Case Histories
Case History 1: Child A
This girl, age 10 years came to us from another hospital. She had been admitted there with a two month history of swelling of the face. The diagnosis of BL had been made on clinical grounds, and chemotherapy with oral cyclophosphamide commenced. This was continued for two weeks in sub-optimal doses. The carers of this child had been counselled that the child's disease had not responded to the treatment, and she was to be discharged as terminally ill. Having heard shortly afterwards that a Trial of treatment for BL was being conducted at BBH, the family travelled to Banso and brought the child to Children's ward, BBH. That was 19th July, 2003.
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Child A; before treatment
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On examination she had gross swellings of all four quadrants of the jaw. Tumour tissue was equally extensive inside the mouth, so that she could scarcely move her mouth, and could neither eat nor drink. Emotionally she was very distressed and distrustful of us.
There was no evidence clinically of disease elsewhere; the abdomen was clear, CNS uninvolved; no lymphadenopathy. FNA of the facial lesions proved the diagnosis of BL. Bone marrow aspiration and lumbar puncture - both under light ketamine anaesthesia - proved the bone marrow and CSF to be free of Burkitt lymphoblasts. Ultrasound examination of the abdomen confirmed that there was unsuspected involvement of kidney, and a small pelvic mass.
We thus were able to diagnose BL of Murphy Stage III (disease on both sides of the diaphragm). Since child A was not naïve to chemotherapy, strictly speaking she was not eligible for the Trial. The object of the Trial was to observe the response to our Protocol chemotherapy unconfounded by the possible effect of any previous chemo. Two things were however obvious to us; first, whatever this child had been given it hadn't worked at all - for she was indeed in the late stages of disease. Second, we came later to learn that the doses of oral cyclophosphamide she had been given were very low. So it was that our overall Co-ordinator [Professor Hesseling] agreed child A be admitted to the Trial, but that her prior low-dose chemo be declared when our results are presented.
The response to IV cyclophosphamide on days 1, 8, and 15, was remarkable and gratifying. A unit of blood was given before the first chemo. From constant crying and misery the child started to sit up and smile, and talk, and eat and drink. After the third chemo she was allowed home for two weeks - after which she was brought back for the fourth chemo. With this and the fifth chemo, we saw a very different, and strong, and spirited patient! Her facial swellings had gone, there was no recurrence anywhere else, and this now lively 10 year old could not understand why she had to be put on a couch again to receive any more needles and injections. She was now a fighter of considerable accomplishment, struggling free of the nurses and escaping from the examination couch.
The situation was different again at her 6th and final chemo. Our patient walked into the ward with arms open and hugged us all. Her happy eyes and wide smile were bewitching; to see that mouth so widely open was in the sharpest possible contrast to the poor tumour-packed mouth of 10 weeks ago. The final treatment having been given, she was discharged - to be seen for follow-up in 6 months.
That appointment was duly kept, and child A was well and free from recurrence; she is among our hopeful cures. Her final assessment for the Trial records, will be in July 2004. She was seen for her 1 year follow-up, and is well and lively, and smiling, and beautiful.
This child reached the hearts of all the ward staff; we will never forget her, nor the happiness of her parents. None of us can remain detached and non-emotional about her. She is a fine example of what can be achieved with simple chemotherapy; our 'gold standard' for African children.
Francine Tchintseme MD, Children's Physician
Nurse Julia Ngoran SRN; Nurse in Charge Children's ward
Banso Baptist Hospital, Kumbo
April 2004
Case History 2: Child S
Tricky transfusions: this is a more technical account than the preceding one. We had hoped to publish it in a medical journal, but the manuscript was rejected! Unlike child A, child S had the BL in his belly, but like her he did well, in spite of the anxiety he caused his doctors and nurses!
A 7-year old boy was admitted to our hospital with a two month history of increasing abdominal swelling. He had been eating well until shortly before admission. There was no vomiting, constipation, diarrhoea or dysuria. There were no facial or other swellings. He was mobile. On examination his general condition was fair; with no wasting, distress, pallor, or fever. The only significant finding was of a large, firm, irregular mass occupying most of the left abdomen. Investigations showed Hb 7.7G/dL; WBC 4.2x10 9 /L; malaria smear negative; Sickle cell test negative; HIV negative. Urinalysis - ketones only; stool microscopy - normal. Ultrasonography of abdomen reported a huge spleen with a complex mass occupying half of the splenic tissue. Liver, kidneys, pancreas - normal. Fine needle aspiration of the mass was positive for Burkitt's lymphoma. Bone marrow and CSF examinations - negative. Our diagnosis was Burkitt's lymphoma, Murphy Stage III (extensive intra-abdominal disease) 1. Thirty minutes after the investigations he had several small haematemeses, totalling 300mL
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Child S; before treatment
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His haemodynamic status remained stable. Crossmatching was done and one unit of blood requested to be held in reserve. Intravenous fluids and allopurinol for tumour lysis prevention were commenced. Tabs. mag trisil and caps. omeprazole were commenced on the assumption the haematemeses represented a stress ulcer. On day 3 of admission we gave the first chemotherapy according to the protocol 2 in use at this hospital. On day 4 the Hb was 6.1G/dL. The laboratory reported that the patient's blood agglutinated all donor blood samples, including that of his mother, and Gp O Rh negative blood in the bank. By day 7 his abdominal mass had almost disappeared.
On day 9 the Hb had dropped to 3.7G/dL There was no further bleeding, no jaundice, and no haemoglobinuria. His general condition was otherwise good. Since our protocol (designed by members of the International Society of Paediatric Oncology) required that chemotherapy be delayed until the Hb > 7G/dL, the search for compatible blood continued, but without success. We assumed his anaemia was autoimmune haemolytic, well-known to occur in lymphoma. Direct Coombs test was negative; indirect was not done.
We commenced high dose steroids (prednisolone 2mg/kg/day). On day 12 the Hb remained at 3.7G/dL. Consulting a clinical haematology text 3 we decided to transfuse using 500 mL of his mother's laboratory incompatible Gp O Rh positive blood. This was done slowly, and monitored closely. There were no adverse effects. On day 14 the Hb had risen to 6.1G/dL, and the next day we proceeded with the delayed second chemotherapy. Knowing that another chemotherapy should yet be given, we anticipated that the Hb would drop again, and so planned a second transfusion. This was done on day 16 after admission, using 500mL of incompatible freshly donated Gp O negative blood from a visiting German medical student. Again there were no adverse effects. On day 17 the Hb was 8.7g/dL, and on day 19 was 10G/dL. The high dose steroid was then reduced. On day 20 he received his belated 3 rd chemotherapy. By now the abdominal mass had almost disappeared. On day 21 our patient went home. He returned for his final chemotherapies.
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The first difficult blood transfusion
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He remains well, has gained weight, and has no abdominal mass..Our dilemma in the resource-poor African setting was clear: Our patient had a highly malignant well- advanced Burkitt's tumour. Chemotherapy showed every chance of rescuing him. Severe anaemia prevented us from proceeding. The laboratory's standard 4-step antibody screening showed agglutination at the early saline-testing stage. His anaemia thus appeared untreatable by transfusion, and was unresponsive to steroids. With 'backs to the wall' we transfused him. The outcome seems to have justified the action.
Faced with aggressive disease, and with the child's best interest in view 4, bold steps may be required - even in the teeth of contrary advice from the laboratory.
We confirm here, that the carers of both of these patients provided written permission for the case histories of their children to be used in any way thought beneficial to the doctors in charge, if it would be likely to benefit other children. The use of photographs of the children was also permitted.
Francine Tchintseme - Children's Physician
Peter McCormick - Children's Physician
Tancho Samuel - Senior Laboratory Technician
Banso Baptist Hospital, Kumbo, N W Province, Cameroon
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Child A; cured
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Child S; cured
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References:
1. S B Murphy; Seminars in Oncology; Vol 7, No. 3 (September) 1980
2. P Kazembe, P B Hesseling, B E Griffin, et al. Long term survival of children with Burkitt's lymphoma in Malawi after cyclophosphamide monotherpy. Med Pediatr Oncol 2003; 40: 23-25
3. Wintrobe's Clinical Hematology; ch 41; Autoimmune hemolytic anemias. John Foerster
4. UN Convention on the Rights of the Child 1989; Article 3
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Thought for the day;
Time was running out for these two children, putting one in mind of Horace (65BC - 8BC): '…dum loquimor, fugit invida aetas; CARPE DIEM*, quam minimum credula postero…'
GRASP THE DAY!
(Burkitt's: 2 cases)
Beryl Thyer Memorial Africa Trust, a UK registered charity ~ 1112603
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