Skip to main content
. Author manuscript; available in PMC: 2018 Feb 1.
Published in final edited form as: Curr Hematol Malig Rep. 2017 Feb;12(1):29–38. doi: 10.1007/s11899-017-0366-1

Table 1.

A Partial List of Studies Detailing Types of Treatment and Response Rates in CLL Patients with Autoimmune Cytopenias

Reference N AIC subtypes Management Response (Complete response = CR, Partial response = PR)*
Alzaki (2014)62 30 16 – AIHA
8 – ITP
5 – Evans
1 – PRCA		 Alkylator based therapy vs. chemo-immunotherapy

  • No difference between two types of therapy (p=0.885)
Bowen (2010)30 20 17 – AIHA
8 – ITP
2 - PRCA		 R-CVP x 2–6 cycles (median 4.9 cycles)

  • 70% CR

  • 25% PR
Carli (2016)74 25 25 – Evans Steroids +/− IVIg in 9/18 patients; chemotherapy +/− rituximab in 9/18 patients

  • Steroids +/− IVIg: 71% response for ITP (42% CR), 87% AIHA (25% CR)

  • Chemotherapy +/− rituximab: 78% response for ITP (67% CR), 100% response for AIHA (38% CR)
Cortes (2001)75 31 16 – Anemia
29 – ITP		 Cyclosporin A at 300 mg/day

  • 63% response in anemia

  • 62% response in ITP
D’Arena (2006)37 14 14 – AIHA Rituximab (375 mg/m2) x 4 cycles

  • 22% CR

  • 50% PR
Gomez-Almaguer (2009)76 11 5 – AIHA
6 – ITP		 Alemtuzumab, rituximab

  • Of 5 AIHA patients, 100% CR, with 1/5 relapse

  • Of 6 ITP patients – 2 (33%) CR, 2 (33%) PR
Gourguechon (2014)77 11 11 – Evans Steroids +/− IVIg – first line; splenectomy = 4; rituximab =4; azathioprine =3, hydroxychloroquine =1, vinkaalcaloids =1

  • 9 (81%) CR with steroids

  • 4 (100%) CR with rituximab, but relapse in 3/4 patients

  • 4 underwent splenectomy, with relapse in 3/4 patients
Gupta (2002)43 8 8 – steroid-refractory AIHA RCD x 4 cycles

  • 8 (100%) achieved remission of AIHA

  • 5 converted to Coombs negative
Kaufman (2009)48 21 18 – AIHA
1 – ITP
2 – Evans		 RCD x 1–9 cycles (median 2.5 cycles)

  • 100% AIHA responded

  • 12 AIHA relapsed; 9 were retreated with RCD and responded

  • 50% converted to Coombs negative
Rossignol (2011)60 48 26 – AIHA
9 – ITP
8 – Evans
5 – PRCA		 RCD x median 4 cycles

  • 89.5% response rate obtained, irrespective of type of cytopenia

  • Relapse in 19 patients (40%)
Michel (2009)78 68 68 – Evans Steroids; 50 required second-line treatment (splenectomy =19, rituximab =11)

  • 22 (32%) CR

  • 16 (24%) died
Sharma (2014)79 22 22 – AIHA RCD in 20 patients or R-CVP in 2 patients x 4 median cycles

  • 9/22 (41%) CR

  • 8/22 (36%) PR

  • Relapse in 4 patients (18%)
*

Treatment response criteria for autoimmune cytopenia14

AIHA and PRCA:

CR = normal hemoglobin

PR = hemoglobin >10 g/dL

NR = hemoglobin < 10 g/dL or no improvement

ITP:

CR = normal platelet count

PR = platelet count ≥ 30 × 109/L

NR = platelet count < 30 × 109/L or no improvement

AIG:

CR = normal neutrophil count

PR = ANC ≥ 1 × 109/L

NR = ANC < 1 × 109/L or no improvement

Abbreviations of regimen:

RCD – Rituximab, cyclosphosphamide, dexamethasone

R-CVP – Rituximab, cyclophosphamide, vincristine, prednisone