Table 1

Patient characteristics

PatientSex (M/F)Age at diagnosis, yearst(11;14)White blood cells ×109/LLHSTherapy*Survival, months
B-PLL 1 61 No 39 No No Yes Chemo 135+ 
B-PLL 2 59 No 30 No No Yes Chemo 
B-PLL 3 70 No 173 No No Yes Chemo 39 
B-PLL 4 84 No 180 No No Yes Chemo 14 
B-PLL 5 49 No 53 Yes Yes Yes Chemo, SCT 199+ 
B-PLL 6 43 No 210 Yes No Yes Chemo 97 
B-PLL 7 69 Yes 30 No No Yes Splen 2.5 
B-PLL 8 74 Yes Yes No Yes Splen, chemo 108 
B-PLL 9 60 Yes 227 No No Yes Splen, chemo 82+ 
B-PLL 10 43 Yes 192 Yes No Yes Splen, chemo 
B-PLL 11 63 Yes 37 No No Yes Splen, chemo 19 
B-PLL 12 52 Yes 241 Yes Yes Yes Chemo 12 
B-PLL 13 44 Yes 116 No Yes Yes Splen, chemo 24 
PatientSex (M/F)Age at diagnosis, yearst(11;14)White blood cells ×109/LLHSTherapy*Survival, months
B-PLL 1 61 No 39 No No Yes Chemo 135+ 
B-PLL 2 59 No 30 No No Yes Chemo 
B-PLL 3 70 No 173 No No Yes Chemo 39 
B-PLL 4 84 No 180 No No Yes Chemo 14 
B-PLL 5 49 No 53 Yes Yes Yes Chemo, SCT 199+ 
B-PLL 6 43 No 210 Yes No Yes Chemo 97 
B-PLL 7 69 Yes 30 No No Yes Splen 2.5 
B-PLL 8 74 Yes Yes No Yes Splen, chemo 108 
B-PLL 9 60 Yes 227 No No Yes Splen, chemo 82+ 
B-PLL 10 43 Yes 192 Yes No Yes Splen, chemo 
B-PLL 11 63 Yes 37 No No Yes Splen, chemo 19 
B-PLL 12 52 Yes 241 Yes Yes Yes Chemo 12 
B-PLL 13 44 Yes 116 No Yes Yes Splen, chemo 24 

Chemo, chemotherapy (fludarabine [8 patients]; fludarabine, cyclophosphamide, rituximab [2 patients]; alemtuzumab [1 patient]; CHOP: cyclophosphamide, doxorubicin, vincristine, prednisone [6 patients]; DHAP: dexamethasone, cytarabine, cisplatin [3 patients]; chlorambucil [3 patients]; deoxycoformycin [1 patient]; high-dose cytarabine [1 patient]); H, hepatomegaly; L, lymphadenopathy;); S, splenomegaly; Splen, splenectomy; SCT, allogeneic sibling stem cell transplantation.

*

Splenectomy was particularly performed in patients diagnosed before 2001, when chemotherapeutic options to reduce spleen size were still limited. Spleen size was comparable between patients diagnosed before or after 2001. Morphological data from the removed spleen were available from patients 7, 8, 10, 11, and 13. B-PLL10 showed disrupted architecture with infiltration of prolymphocyte-like cells in particularly the red pulpa. B-PLL7 and B-PLL8 showed disrupted architecture with infiltration of MCL-like cells (either classical or monocytoid). B-PLL11 showed normal splenic architecture without clear leukemic infiltration. B-PLL13 showed normal splenic architecture with focal infiltration of atypical lymphocytes (some with a clear nucleolus), also in the red pulpa.

+, Still alive at last follow-up (2013).

Subsequently lost to follow-up.

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