BACKGROUND: The differential diagnosis of BCLD in patients (pts) presenting with splenomegaly as the sole clinical finding is extremely difficult, due to the lack of lymph node histology. The diagnosis is based on morphology and immunophenotype (IF) from the blood and/or bone marrow. However, the reproducibility of morphology is limited and there is considerable overlap in IF findings. AIMS: The comparative analysis of pts with BCLD presenting with sole splenomegaly.

METHODS: All pts presented to our Unit with a probable BCLD and splenomegaly as the sole clinical finding, between 1980 and 2005 were retrospectively analyzed. Pts were classified into 4 groups according to their IF: Group A: CD5+/CD23+ [B-CLL phenotype], Group B: CD5+/CD23− [Mantle cell lymphoma phenotype], Group C: CD5−/CD23+, and Group D: CD5−/CD23− [Groups C and D: Splenic marginal zone lymphoma phenotype]. Hairy cell leukemia cases were excluded from the analysis. Pts’ characteristics, first line treatment, disease specific survival (DSS), freedom from 1st treatment (FF1T)and freedom from 2nd treatment (FF2T) were analyzed.

RESULTS: 71 pts (41 males) with a median age of 63 years (range: 39–83 y) were recorded. The 4 groups differed significantly in all their main characteristics, except of age, platelet count and frequency of lymphocytosis as shown in the table. 6 pts have not received any treatment, 27 were splenectomized, 16 received chlorambucil, 11 Rituximab and the remaining 11 other first-line treatments. The median follow up of the currently alive pts is 44.7 months (3–303). 34 pts have not required 2nd therapeutic intervention. The 10-year DSS of all 71 pts was 71±7%. The 10-year DSS and 5-year FF2T did not differ significantly between the 4 groups. However group B had a worse DSS (p=0.05) and FF2T (p=0.0036) compared to all other groups together. Cases who were CD38+ (p=0.055), CD11c− (p=0.001) and had Hb ≤12g/dL (p=0.03) had a worse prognosis.

CONCLUSIONS: Pts with BCLD and splenomegaly can be classified into different clinicopathologic entities according to basic IF characteristics. CD5+/CD23− splenomegalic BCLDS seem to have a worse outcome. CD38+, CD11c− and anemia are poor prognostic features. The best therapeutic strategy remains to be established.

Characteristics of the 4 Splenomegalic Groups

Group AGroup BGroup CGroup Dp
N 19 16 20 16  
Spleen (cm) 14 (6–20) 16 (9–34) 10 (5–30) 10 (4–20) 0.03 
Hb (g/dL) 12.0 11.0 10.0 12.4 0.02 
WBC (×10 ^9/L) 30 10.8 10.1 10.8 0.02 
CD38+ 28% 75% 5% 6% <0.001 
CD25+ 63% 38% 19% 0% 0.002 
CD11c+ 56% 27% 47% 80% 0.03 
Group AGroup BGroup CGroup Dp
N 19 16 20 16  
Spleen (cm) 14 (6–20) 16 (9–34) 10 (5–30) 10 (4–20) 0.03 
Hb (g/dL) 12.0 11.0 10.0 12.4 0.02 
WBC (×10 ^9/L) 30 10.8 10.1 10.8 0.02 
CD38+ 28% 75% 5% 6% <0.001 
CD25+ 63% 38% 19% 0% 0.002 
CD11c+ 56% 27% 47% 80% 0.03 
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Topics:
lymphoproliferative disorders, splenomegaly, brachial plexus neuritis, abnormal involuntary movement scale, alberta infant motor scales, anemia, chlorambucil, follow-up, hairy-cell leukemia, immunophenotyping

Disclosure: No relevant conflicts of interest to declare.

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Corresponding author

2006, The American Society of Hematology
2006
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