Table 1.

Clinical characteristics of CLL patients diagnosed with RS

Patient	1	2	3	4
At the time of CLL diagnosis
Age, y	51	69	65	71
Sex	Female	Male	Male	Female
WBC ×109/L	57	4.9	28.4	9.6
Hemoglobin, g/dL	14	13.4	12.5	12
Platelet count ×109/L	370	197	155	218
β2 microglobulin, mg/dL	1.72	1.91	N/A	N/A
Rai stage	I	I	0	I
IGHV mutation status	Unmutated	Unmutated	Unmutated	Unmutated
IGHV4-39 gene usage	No	No	Yes	No
ZAP-70 expression ≥20%	Yes	Yes	Yes	Yes
CD49d expression ≥30%	No	No	No	Yes
CD38 expression ≥30%	No	No	N/A	No
FISH	del13q	Normal	Normal	del17p
CLL therapy	1. Rituximab, alemtuzumab, and β-glucan 2. Pentostatin, cyclophosphamide, and rituximab 3. Everolimus and alemtuzumab	1. Bendamustine and rituximab	1. Pentostatin, cyclophosphamide, and rituximab	1. Bendamustine and rituximab 2. Everolimus and alemtuzumab 3. Methylprednisolone and ofatumumab 4. Methylprednisolone and alemtuzumab
Time from last CLL therapy to RS diagnosis (months)	8	2	24	<1
At the time of DLBCL diagnosis
Age, y	62	74	68	74
WBC ×109/L	131.6	80.3	6.3	2.6
Hemoglobin, g/dL	10.8	9.2	14.5	9.8
Platelet count ×109/L	116	111	149	169
LDH, U/L (normal:111-222 U/L)	810	236	204	466
RS prognosis score4	1 (low)	2 (low-intermediate)	0 (low)	3 (high-intermediate)
Type of biopsy specimen	Bone marrow aspirate and biopsy	Core biopsy of T7 bone	Left neck excisional biopsy	Bone marrow aspirate and biopsy
Subtype (GCB vs ABC)	ABC	ABC	ABC	ABC
MYC rearrangement by FISH	Negative	Not done	Not done	Negative
EBV status	N/A	N/A	N/A	Negative
Initial therapy for RS	1. R-CHOP (2 cycles, PR) 2. R-ICE (1 cycle, PD) 3. Palliative therapy with methylprednisolone (twice per wk for 6 wks, SD)	1. R-CHOP (2 cycles, PD) 2. R-EPOCH (2 cycles, PD)	1. R-CHOP (2 cycles, PD) 2. R-ICE (3 cycles, PR) 3. R-DHAP (2 cycles, PD)	None
Ibrutinib dose, mg per day	420	140	420	420
Concomitant therapy with ibrutinib	Methylprednisolone 1000 mg Intravenously (twice per wk for 1 mo, once per wk for 1 mo, and once every 2 wks for 1 mo)	Methylprednisolone 1000 mg Intravenously (twice per wk for 2 mo)	None	None
Duration of ibrutinib therapy, months	8.5	3.7	2.8	10.8
Best response achieved	PR	Clinical benefit*	CR	PR
Status at most recent follow-up	Progressive DLBCL	Death	Ongoing complete remission	Progressive CLL

EBV, Epstein-Barr virus; FISH, fluorescence in-situ hybridization; IGHV, immunoglobulin heavy chain genes; LDH, lactate dehydrogenase; N/A, not available; PD, progressive disease; PR, partial response; SD, stable disease; WBC, white blood cell count; ZAP-70, zeta-chain–associated protein kinase 70.

^*Documentation of reduction in lymphadenopathy by physical examination. The patient died of pulmonary mucormycosis (diagnosed prior to ibrutinib initiation) after 15 weeks of therapy and prior to repeat imaging to allow for response assessment.