|dc.description.abstract||Substantial geographical differences exist for Hodgkin and other lymphoproliferative disorders
with these having previously been documented in a report from the lymphoma reclassification
project. In the light of rampant human immunodeficiency syndrome, largely
centred in sub-Sahara, this experience is updated in a further 512 consecutive individuals
treated over an 8-year period in a privately based academic centre.
Median age was 55.2 years 61% were males, 10% had Hodgkin lymphoma and, overall,
constitutional symptoms were present in 20%. Prior to referral 19% had received chemotherapy
and a further 20% some form of irradiation.
Median survival in hairy cell leukaemia (n = 14), chronic lymphocytic leukaemia–small
lymphocytic lymphoma (n = 103), Hodgkin (n = 41) and follicular lymphoma (n = 59) was
not reached at the time of analysis and exceeded 36 months. This was followed by
32 months for those with mantle cell (n = 7), splenic (n = 2) and extranodal marginal cell
(n = 11), 24 months for T-cell lymphomas (n = 24), 20 months for diffuse large B-cell variants
(n = 88) but only 12 months for the aggressive tumours exemplified by Burkitt (n = 7)
and lymphoblastic subtypes (n = 6). The remaining 36 patients had to be excluded because
numbers were too small for statistical analysis or unreliable staging. Adverse factors were
constitutional symptoms, prior treatment with chemotherapy, intermediate or high-risk
scores as defined by the international prognostic index, histologic grading and certain anatomical
sites of primary tumour. In contrast gender, staging by Rye or Rai classification, retroviral
infection and prior treatment with radiotherapy were without effect.
Overall survival at 3 years in each category was compared to the curve for the entire
cohort and was 100% in hairy cell leukaemia receiving two chlorodeoxyadenosine and
greater than 88% in Hodgkin lymphoma treated according to the German study group protocols
(p = 0.0004). Corresponding figures for chronic lymphocytic leukaemia–small lymphocytic
lymphoma were 82% (p = 0.0006), follicular lymphoma 71% (p = 0.060),
peripheral T-cell lymphoma 43% (p = 0.0156), diffuse large B-cell lymphoma 39%
(p < 0.0001), aggressive tumours 25% (p = 0.0002) and for the indolent categories including
mantle cell, splenic and extra nodal marginal cell lymphomas 22% (p = 0.2023).
Outcome argues in favour of patient management by a multidisciplinary team implicit in
which are standardised protocols for diagnosis, staging and treatment. Under these circumstances
the well recognized centre effect applies when results approximate those from first
world reference centres. Conversely any deviation from such a disciplined approach is unlikely
to achieve comparable benefit and therefore to be strongly discouraged||en_ZA