hiv-associated b cell all

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British Journal of Haernatology. 1988, 69, 287-289 Case Reports HIV-ASSOCIATED B CELL ALL We read with interest the case report of acute lymphoblastic leukaemia of B cell origin in an anti-HIV positive intravenous drug abuser reported by Rossi et a1 (1988). We believe the followingcase lends further support for the inclusion of B-cell lymphoblastic leukaemia (&ALL) occurring in HIV antibody positive individuals into the CDC definition of AIDS. Unlike the report of Rossi et a1 (1988). whose patient died of pneumocystis carinii pneumonia, the case reported below would not otherwise fulfill the current CDC definition of AIDS. A previously fit 30-year-old male homosexual, who was known to be HIV antibody positive, presented with a short history of malaise, night sweats and bone pain. Examination revealed generalized lymphadenopathy with an enlarged liver (5 cm) and a tender, enlarged spleen (8 cm). Investiga- tion showed a Hb of 10 g/dl, WBC 38.5 x 10y/l (predomin- antly primitive lymphoid cells) and platelets 24 x 10y/l. The bone marrow aspirate was markedly hypercellular with greater than 99% lymphoid blasts of FAB L3 morphology, a minority of the blasts had coarse, granular PAS staining. Lymphocyte phenotyping demonstrated this to be a B-cell ALL. 80% of the cells examined were blasts with weak CALLA (CD10) and TdT positivity, stronger surface staining for B cell related antigens by B4 (CD19). B1 (CD20) and BA1 (CD24). The cells were negative for T cell and myeloid markers. (Marker studies performed by Dr P. Amlot, Department of Immunology, Royal Free Hospital.) Cytogenetic analysis was unsuccessful. CSF showed a slight increase in lymphocytes but no definite blast cells. The blast cells were negative for Epstein-Barr Nuclear Antigen (EBNA). The patient was commenced on a UKALL Xa (adult) chemotherapy schedule. There was an initial reduction in the peripheral blood count and resolution of lymphadenopathy, though matters were complicated by splenic rupture necessi- tating emergency laporotomy. By day 14, however, the disease had recurred and in addition he now developed numerous cranial nerve palsies. CSF examination revealed NEUTROPENIA ACCOMPANYING ERYTHROID APLASIA Human parvovirus (HPV) is known to cause transient failure of erythropoiesis in cases of chronic haemolytic anaemia. Most case reports are of sickle cell disease (Pattison et al. 1981) but the same effect has been seen in hereditary spherocytosis and B thalassaemia intermedia (Rao et al. 1983). We report a case of agranulocytosis and thrombocy- topenia in a transfusion-dependent thalassaemic associated with HPV infection. A British born 19-year-old male thalassaemic of a Turkish Cypriot family has been attending this hospital for regular red leukaemic blasts, and bone marrow examination showed continuing heavy infiltration by leukaemia cells. Treatment was changed to a MACHO regime (Royal Free Hospital) which includes twice weekly intrathecal treatment with cytosine and/or methotrexate. On day 26 of this regime, his bone marrow and CSF were free of blasts indicating remis- sion. A few days later he developed a left VII nerve palsy and lower motor neurone weakness in the left leg. With this evidence of active neuro-leukaemia and because of the high risk of early bone marrow relapse in B-cell ALL, he was given further myeloablative chemotherapy and cranio-spinal irra- diation followed by autologous bone marrow rescue. He tolerated this poorly, developing a high fever which did not respond to treatment and he died 10 weeks after presen- tation. High grade B cell lymphoma in an HIV antibody positive patient is diagnostic of AIDS (Ziggler, 1984)and we are of the opinion that the current CDC classification of AIDS should be widened to include B-cell ALL in HIV seropositive subjects. Department of Haematology, PETER FLANACAN St Stephen’s Hospital, VIJAY CHOWDHURY London, S.W.6 CHRISTINE COSTELLO REFERENCES Centres for Disease Control, US Department of Health and Human Services,Atlanta, Georgia (1986) Classificationsystem for human T-lymphotropic virus type III/lymphadenopathy-associated virus infections. Annals of Internal Medicine. 105, 234-237. Rossi, G., Gorla, R., Cadeo, G.P.. Stellini, R. & Marnone, G. (1988) Acute lymphoblastic leukaemia of &cell origin in an anti-HIV positive intravenous drug abuser. British Journal of Haematology, 65, 140-141. Ziggler. J.L., Beckstead, J.A. et al, (1986) Non-Hodgkin’s lymphoma in 90 homosexual men. New England Journal of Medicine, 311, 565-570. IN HUMAN PARVOVIRUS INFECTION cell transfusions since the age of 4 years. On 5 May 1987 he received a routine monthly transfusion of 5 units after which his haemoglobin was 9.8 g/dl, his white cell count 5.3 x lo9/ 1 and his platelet count 267 x 109/l. Nine days later he was admitted via casualty complaining of 3 d offlu-like illness and 1 d of a severe sore throat. He was found to be pyrexial at 39OC with a reddened throat and his blood count revealed a haemoglobin of 8.6 g/dl, a white cell count of 1.3 x 109/1 (neutrophils 0.55 x 109/l. lymphocytes 0.52 x 109/1, eosino- phils 0.06 x 10y/l. basophils 0.04 x 109/1, monocytes 287

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Page 1: HIV-ASSOCIATED B CELL ALL

British Journal of Haernatology. 1988, 69, 287-289

Case Reports

HIV-ASSOCIATED B CELL ALL

We read with interest the case report of acute lymphoblastic leukaemia of B cell origin in an anti-HIV positive intravenous drug abuser reported by Rossi et a1 (1988). We believe the following case lends further support for the inclusion of B-cell lymphoblastic leukaemia (&ALL) occurring in HIV antibody positive individuals into the CDC definition of AIDS. Unlike the report of Rossi et a1 (1988). whose patient died of pneumocystis carinii pneumonia, the case reported below would not otherwise fulfill the current CDC definition of AIDS.

A previously fit 30-year-old male homosexual, who was known to be HIV antibody positive, presented with a short history of malaise, night sweats and bone pain. Examination revealed generalized lymphadenopathy with an enlarged liver (5 cm) and a tender, enlarged spleen (8 cm). Investiga- tion showed a Hb of 10 g/dl, WBC 38.5 x 10y/l (predomin- antly primitive lymphoid cells) and platelets 24 x 10y/l. The bone marrow aspirate was markedly hypercellular with greater than 99% lymphoid blasts of FAB L3 morphology, a minority of the blasts had coarse, granular PAS staining. Lymphocyte phenotyping demonstrated this to be a B-cell ALL. 80% of the cells examined were blasts with weak CALLA (CD10) and TdT positivity, stronger surface staining for B cell related antigens by B4 (CD19). B1 (CD20) and BA1 (CD24). The cells were negative for T cell and myeloid markers. (Marker studies performed by Dr P. Amlot, Department of Immunology, Royal Free Hospital.) Cytogenetic analysis was unsuccessful. CSF showed a slight increase in lymphocytes but no definite blast cells. The blast cells were negative for Epstein-Barr Nuclear Antigen (EBNA).

The patient was commenced on a UKALL Xa (adult) chemotherapy schedule. There was an initial reduction in the peripheral blood count and resolution of lymphadenopathy, though matters were complicated by splenic rupture necessi- tating emergency laporotomy. By day 14, however, the disease had recurred and in addition he now developed numerous cranial nerve palsies. CSF examination revealed

NEUTROPENIA ACCOMPANYING ERYTHROID APLASIA

Human parvovirus (HPV) is known to cause transient failure of erythropoiesis in cases of chronic haemolytic anaemia. Most case reports are of sickle cell disease (Pattison et al. 1981) but the same effect has been seen in hereditary spherocytosis and B thalassaemia intermedia (Rao et al. 1983). We report a case of agranulocytosis and thrombocy- topenia in a transfusion-dependent thalassaemic associated with HPV infection.

A British born 19-year-old male thalassaemic of a Turkish Cypriot family has been attending this hospital for regular red

leukaemic blasts, and bone marrow examination showed continuing heavy infiltration by leukaemia cells. Treatment was changed to a MACHO regime (Royal Free Hospital) which includes twice weekly intrathecal treatment with cytosine and/or methotrexate. On day 26 of this regime, his bone marrow and CSF were free of blasts indicating remis- sion. A few days later he developed a left VII nerve palsy and lower motor neurone weakness in the left leg. With this evidence of active neuro-leukaemia and because of the high risk of early bone marrow relapse in B-cell ALL, he was given further myeloablative chemotherapy and cranio-spinal irra- diation followed by autologous bone marrow rescue. He tolerated this poorly, developing a high fever which did not respond to treatment and he died 10 weeks after presen- tation.

High grade B cell lymphoma in an HIV antibody positive patient is diagnostic of AIDS (Ziggler, 1984) and we are of the opinion that the current CDC classification of AIDS should be widened to include B-cell ALL in HIV seropositive subjects.

Department of Haematology, PETER FLANACAN St Stephen’s Hospital, VIJAY CHOWDHURY London, S.W.6 CHRISTINE COSTELLO

REFERENCES

Centres for Disease Control, US Department of Health and Human Services, Atlanta, Georgia (1986) Classification system for human T-lymphotropic virus type III/lymphadenopathy-associated virus infections. Annals of Internal Medicine. 105, 234-237.

Rossi, G., Gorla, R., Cadeo, G.P.. Stellini, R. & Marnone, G. (1988) Acute lymphoblastic leukaemia of &cell origin in an anti-HIV positive intravenous drug abuser. British Journal of Haematology, 65, 140-141.

Ziggler. J.L., Beckstead, J.A. et al, (1986) Non-Hodgkin’s lymphoma in 90 homosexual men. New England Journal of Medicine, 311, 565-570.

IN HUMAN PARVOVIRUS INFECTION

cell transfusions since the age of 4 years. On 5 May 1987 he received a routine monthly transfusion of 5 units after which his haemoglobin was 9.8 g/dl, his white cell count 5.3 x lo9/ 1 and his platelet count 267 x 109/l. Nine days later he was admitted via casualty complaining of 3 d offlu-like illness and 1 d of a severe sore throat. He was found to be pyrexial at 39OC with a reddened throat and his blood count revealed a haemoglobin of 8.6 g/dl, a white cell count of 1.3 x 109/1 (neutrophils 0.55 x 109/l. lymphocytes 0.52 x 109/1, eosino- phils 0.06 x 10y/l. basophils 0.04 x 109/1, monocytes

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