Search

Background. A role for genetic factors in chronic lymphocytic leukemia (CLL) is unequivocal based on evidence from multiply affected families, from case series, twin and case-control studies, and population-based registry studies. Similar characteristics have been observed for non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL). Population-based studies have found CLL, NHL and HL to co-occur in families. A few hospital-based series have explored survival outcomes in familial vs. sporadic CLL patients based on the hypothesis that genetic mechanisms involved in the causation of familial lymphomas might influence survival, however these have been based on small numbers with inconsistent findings. We have conducted a large study in Scandinavia including more than 41,000 lymphoma patients to quantify survival outcomes among familial vs. sporadic patients. Methods. We used the population-based central Cancer and Multigenerational Registries in Sweden and Denmark to identify all CLL (n=7749), NHL (n=25801), and HL (n=7476) patients diagnosed 1958–2001, with linkable first-degree relatives. All relatives were linked with the Cancer Registries to obtain information on CLL, NHL, and HL. Using Cox proportional hazard models, we calculated hazard ratios (HRs) and 95% confidence intervals (CIs) as measures of overall survival for CLL, NHL, and HL patients with vs. without family history of lymphoma. Results. We found 85 (1.1%) CLL, 207 (0.8%) NHL, and 96 (1.3%) HL patients with family history of lymphoma. Overall survival was similar for CLL (HR=1.24, 95% CI 0.93–1.67), NHL (HR=0.97, 95% CI 0.80–1.16), and HL (HR=0.78, 95% CI 0.51–1.19) patients with vs. without family history of lymphoma. Risk-estimates were similar when we calculated overall survival by age at lymphoma diagnosis (above vs. below median age), year at diagnosis (before vs. after 1987), and by sex. Consistent with the literature, including all CLL, NHL and HL patients, older age (p Conclusions: Survival patterns were similar for CLL, NHL, and HL patients with vs. without family history of lymphoma, suggesting that familial lymphomas do not have an altered clinical course. Overall, there is no evidence at this time to modify therapeutic strategies for patients with CLL, NHL, or HL based solely on family history.

Background: Appropriately aggressive treatment of haematological malignancies can be complicated by a variety of life threatening events. Despite high hospital mortality rates for such patients admitted to intensive care units (ICU) it is now generally considered to be appropriate to offer intensive care to selected cases, provided there is a reasonable prospect of cure or at least worthwhile palliation. Aims and Methods: We conducted a retrospective observational study to assess outcome and prognostic indicators in consecutive patients with hematological diseases admitted to the ICU during a 6-year-period. Results: From 1996 through 2001, a total of 95 patients with hematological diseases and a median age of 57 years (range 16–86) were admitted to the ICU. The median duration of ICU stay was 1 day (mean 4.2 days: range 1–67 days). The Mean Acute Physiology and Chronic Health Evaluation II (APACHE II) score was 20 (± 9). The large majority of patients underwent active treatment of a hematological malignancy (90%) with acute leukaemia (27%), non-Hodgkin lymphoma (24%) and multiple myeloma (10%) dominating. Respiratory failure (46%), sepsis (24%), cardiovascular complications (9%) and bleeding disorders (7%) were the major reasons for ICU admission. A total of 49 patients (51%) had a microbiologically verified infection and 21 (22%) had bacteremia. Crude ICU, 4-week and 6-month mortality rates were 28%, 45%, and 57%, respectively. An APACHE II score > 30 predicted a high short-term mortality rate (p= 0.0001). However, age > 65 years, respiratory failure, bacteremia, and a diagnosis of acute leukemia were not significantly associated with a poor short-term survival (p> 0.05). A total of 30 patients (31%) were alive after a minimum follow up of 3.5 years. Conclusion: The lower mortality rate as compared with most other series is probably explained by a more liberal attitude towards ICU admission. Not withstanding this, for a substantial proportion of critically ill hematological patients a short time care at an ICU is life saving. Patients with life threatening complications of haematological disease should be offered intensive care unless or until it is clear that there is no prospect of recovery from the acute illness or that the underlying malignancy cannot be controlled.