A CAJM study on Lymphoproliferative malignancies observed at a teaching hospital in South Africa among  black Africans.The association of classical Kaposi’s sarcoma with lymphoproliferative disorders is well known. However, far less is known about lymphoproliferative malignancies in endemic African Kaposi’s sarcoma. A review of 47 patients with the endemic type of Kaposi’s sarcoma treated at the Johannesburg Teaching Hospital Complex between 1980 and 1992 revealed four patients (8,5 pc) in whom Kaposi’s sarcoma was associated with a malignant lymphoma. Possible pathogenetic mechanisms are suggested and the current literature is reviewed

Ruff, P.

Spencer, D.

Stein, M.E.

Central African Journal of Medicine

English

IDS OpenDocs

-CENTRAL AFRICAN 
JOURNAL 
OF
MEDICINE
Vol. 40, No. 7 CONTENTS July, 1994
ORIGINAL ARTICLES
Reduced semen quality and risk behaviour 
amongst men consulting a referral STD clinic....
Lymphoproliferative malignancies in association 
with endemic African Kaposi’s Sarcoma.............
Determination of appropriate clomipramine 
dosage among depressed African outpatients in 
Dar es Salaam, Tanzania......................................
Bronchoscopic extraction of aspirated foreign 
bodies in children in Harare Central Hospital, 
Harare, Zimbabwe.................................................
Importance of inter-sectoral co-ordination in the 
control of communicable diseases, with special 
reference to plague in Tanzania............................
CASE REPORTS
Prolapsed pedunculated sub-mucous uterine 
fibroleiomyomata at the University Teaching 
Hospital, Lusaka....................................................
REVIEW ARTICLES
Advances in electrodiagnosis in Zimbabwe. 
Part I: Peripheral nerve conduction studies and 
electromyography..................................................
MT Mbizvo, AP Danso, S Tswana, M Bassett,
E Marowa, L Mbengeranwa.................................. 170
ME Stein, D Spencer, P Ruff................................ 176
GP Kilonzo, SF Kaaya, JK Rweikiza, M Kassam,
GM oshi,................................................................. 178
AA Ahmed............................................................. 183
BS Kilonzo............................................................. 186
NC Sikazwe........................................................... 192
O Parry, J Mielke, LF L evy................................. 195
SUPPORTED BY CEV1AS MEDICAL AID SOCIETY
CENTRAL AFRICAN
JOURNAL OF MEDICINE
Vol. 40, No. 7 ,1994
Lymphoproliferative 
malignancies in association 
with endemic African 
Kaposi’s Sarcoma
ME STEIN, D SPENCER, P RUFF 
SUMMARY
The association of classical Kaposi’s sarcoma with 
lymphoproliferative disorders is well known. How­
ever, far less is known about lymphoproliferative ma­
lignancies in endemic African Kaposi’s sarcoma. A 
review of 47 patients with the endemic type of 
Kaposi’s sarcoma treated at the Johannesburg Teach-
Department of Medicine
Division o f Clinical Haematology and Medical Oncology
University ofWitwatersrand Medical School
7 York Road
Parktown 2193
Johannesburg, South Africa
Correspondence to:
P Ruff
ing Hospital Complex between 1980 and 1992 re­
vealed four patients (8,5 pc) in whom Kaposi’s sar­
coma was associated with a  malignant lymphoma. 
Possible pathogenetic mechanisms are suggested and 
the current literature is reviewed.
INTRODUCTION
The close association between classical Kaposi’s sar­
coma (CKS) and lymphoproliferative malignancies 
(LPM) has been reported with a frequency of between 
17 pc and 60 pc.1 More recent studies have reported 
LPMs in up to one third of the patients with CKS .2Most 
lymphomas are of B-cell origin including chronic lym­
phocytic leukaemia, multiple myeloma, follicular and 
diffuse lympomas,3 and appear to be associated with 
impaired immunological function.44
There were very few reports of the association be­
tween the endemic, African variant of Kaposi’s sar­
coma (AKS) and LPMs.1 The scarcity of reports may, 
however, be due to socio-economic factors including 
lack of adequate medical facilities, poor compliance 
and follow up, as well as increased mortality and 
morbidity due to associated infectious events.
In this analysis, four patients with LPMs associated 
with AKS are analyzed.
MATERIALS AND METHODS
The records of all patients with AKS treated and 
followed at the Johannesburg Hospital Complex be­
tween 1980 andl992 were analyzed. On presentation 
the patients underwent physical exam ination, 
haematological and biochemical studies and chest radi­
ography. When clinically indicated, further gastro­
intestinal and radiological investigations were per­
formed. All patients were treated with either 
radiotherapaeutic or chemotherapeutic regimens as 
indicated.
RESULTS
Patient characteristics: Forty seven southern African 
(South Africa, Mozambican, Botswanan, Namibian 
and Malawian) patients were included in the study. All 
were male with a mean age of 53 years (range 24-82 
years) and were HIV negative.
Associated lymphoproliferative malignancies: 
Three patients presented with generalised lymphade- 
nopathy simultaneously with AKS. Lymph node bi­
opsy revealed a malignant lymphoma in all three.
176
CENTRAL AFRICAN
JOURNAL OF MEDICINE
Vol. 40, No. 7,1994
namely peripheral T-cell lymphoma, follicular 
lymphoma and immunoblastic lymphoma. In all three 
patients, the lymphoma was limited to the lymph nodes 
and responded to chemotherapy. No specific treatment 
was given for the AKS.
The fourth patient presented simultaneously with 
Kaposi’s sarcoma involving peripheral lymph nodes, 
as well as hepatosplenomegaly, ascites and pleural 
effusion. Haematological and biochemical studies re­
vealed a pancytopenia and pathological liver function 
tests. Immune function studies showed an absolute T- 
cell lymphopenia and a low T4/T8 ratio (0,78:1). Bone 
marrow trephine biopsy demonstrated infiltration with 
a B-cell lymphoma. Following cytarabine (cytosine 
arabinoside) and interferon-alpha therapy, a signifi­
cant reduction in tumour bulk was achieved together 
with a marked symptomatic improvement. The patient 
was then continued on CHOP (cyclophosphamide, 
epirubicin, vincristine and prednisone) with further 
response, albeit of short duration.
The patient relapsed and died three months after the 
start o f CHOP therapy with massive hepatos­
plenom egaly, pancytopenia and extensive 
lymphomatous infiltration of bone marrow.
DISCUSSION
Early reports from the 1960s suggested an association 
between AKS and lymphoproliferative malignancies. 
However, only a few sporadic cases have been re­
ported.810 One case of Hodgkin’s disease was de­
scribed among 19 autopsy cases of AKS.10 Other au­
thors911'13 described sporadic cases of AKS associated 
with non-Hodgkin’s lymphoma, Hodkin’s disease or 
chronic lymphocytic leukaemia. Larger series, com­
prising hundreds of patients with AKS revealed very 
few cases of associated LPM.78
It is possible that the younger age of AKS patients, 
shorter survival due to accompanying infectious dis­
eases and the lack of adequate medical facilities and 
follow up may be responsible for the lower frequency 
of LPM reported in AKS.1416
The aetiology of AKS and associated lymphoproli­
ferative disorders is not entirely clear. AKS is not 
associated with overt immunosuppression.15 Recent 
reports, however, have demonstrated an alteration in 
cell mediated immunity and lymphocytic dysfunction 
in AKS.1819 In Africa, the coexistence of protein-calo­
rie malnutrition as well as tropical and chronic infec­
tions may also result in impaired cellular immunity. T- 
cell regulatory dysfunction leading to unopposed pro­
liferation of abnormal B-cells may also play a role.19 In 
addition, the use of alkylating agents such as nitrogen 
mustard trenimon and cyclophosphamide in AKS may 
also cause immunosuppressive changes and result in 
the emergence of secondary lymphoproliferative ma­
lignancies.20-22 This does not however, apply to our 
study as all four patients presented simultaneously with 
AKS and a malignt lymphoma, and did not receive 
specific treatment for their AKS.
In conclusion therefore, we have described four (8,5 
pc) patients out of a total of 47 patients with AKS, who 
presented simultaneously with a LPM. Although no 
specific aetiology is known, abnormal T-cell and B- 
cell function in AKS, associated with poor nutrition 
and chronic infections may play a role. Improved 
outcome and prolonged survival among patients with 
AKS may result in increased awareness and more 
diagnoses of associated LPMs.
REFERENCES
1. Safai B. Kaposi’s sarcoma: a review of the 
classical and epidemic forms. Ann NY Acad Sci 
1982;437:373-82.
2. Friedman-Kien AE.Saltzmann A. Clinical mani­
festations of classical, endemic African and 
epidemic AIDS associated Kaposi’s sarcoma. J  
Acad Dermatol 1990;22:1237-50.
3. Ulbright TM, Santa-Cruz DJ. Kaposi’s sar­
coma: relationship with haematologic, lymphoid 
and thymic neoplasia. B rJ Cancer 1981 ;47:963- 
73.
4. Friedman-Bimbaum R, Weltfreund S, Katz I. 
Kaposi’s sarcoma: retrospective study of 67 
cases with the classical form. Dermatologica 
1990;180:12-7.
5. Friedman-Bimbaum R, Weltfreund S, Pollack 
S. Classic Kaposi’s sarcoma: T-lymphocyte sub­
sets, T4/T8 ratio and NK cell activity. J  Am 
AcadDermat 1991;24:937-40.
6. Safai B, Mike V.GiraldoB, Beth E, Good RA. 
Association of Kaposi’s sarcoma with second 
primary malignancies: possible etiopathogenic 
implications. Cancer 1980;45:1472-9.
7. Piette MM. The incidence of second malignan­
cies in subsets of Kaposi’s sarcoma. J  Am Acad 
Dermat 1987;16:855-61.
8. Slavin GH, Cameron HM, Singh H. Kaposi’s 
sarcoma in mainland Tanzania: a report of 117 
cases. Br J  Cancer 1969;23:349-57.
177
CENTRAL AFRICAN
JOURNAL OF MEDICINE
Vol. 40, No. 7,1994
9. TaylorJF,SmithPG,BullD,PikeMC.Kaposi’s 
sarcoma in Uganda: geographic and ethnic dis­
tribution. Br J  Cancer 1972;26:483-97.
10. LotheF, Murray JF. Second primary neoplasms 
associated with Kaposi’s sarcoma in African 
patients -  an autopsy report Acta Clin Int Can­
cer 1962;18:100-16.
11. McKiney B . Coincidence of Kaposi’s sarcoma 
and lymphoma -  a case report. E Afr Med J  
1952;42:3-5.
12. Uys CJ, Bennet MB. Kaposi’s sarcoma and 
coincident Hodgkin’s disease in an African 
patient. SAJirJM ed Sci 1959;5:39-41.
13. Kungu A, Gatei DG. Kaposi’s sarcoma in 
Kenya. A retrospective clinicopathological 
study. Antibiotics Chemother 1981;29:38-55.
14. Desmond-Hellmann SD, Katongole-Mbidle E. 
Kaposi’s sarcoma: recent developments. AIDS 
1991 ;5 (Supp. 1): 135-42.
15. Kestens L, MelbyeM, Biggar RJ, Stevens WJ, 
Piot P, De-Muynck A, Taelman H, De-Feyter 
M, Paluku L, Gigase PL. Epidemic Kaposi’s 
sarcoma is not associated with immunodefi­
ciency. Int J  Cancer 1985;36:49-54.
16. Biggar RJ. The AIDS problem in Africa. Lancet 
1986;1:79-82.
17. Stickler MC, Friedman-Kien AE. Kaposi’s sar­
coma. ClinDemuit 1991;9:39-47.
18. Lulat AGMJ. African Kaposi’s sarcoma. Trans 
R Soc Trop Med Hyg 1989;83:1-2.
19. Mairing C, Fiorini R, Boneschi B, Melatti E, 
BrambillaL. Immunologic and immunogenetic 
features of primary Kaposi’s sarcoma. Cancer 
1985;55:1899-901.
20. Cook J. Treatment of Kaposi’s sarcoma with 
nitrogen mustard. Acta Clin Intern Cancer 1962; 
18:494-501.
21. Vogel CL, Templeton CJ, Templeton AC, Tay­
lor JF, Kyalwazi SK. Treatment of Kaposi’s 
sarcoma with actinomycin-D and cyclophos­
phamide: Results of a randomized clinical tri­
als. Int J  Cancer 1975;8:136-43.
22. Kywalazi SK. Chemotherapy of Kaposi’s sar­
coma: experience with trenimon. E Afr Med J  
1968;45:17-26.
178
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Lymphoproliferative malignancies in association with endemic African Kaposi’s Sarcoma

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Lymphoproliferative malignancies in association with endemic African Kaposi’s Sarcoma

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