|
| Study | Year of publication [ref] | Number of patients | Associated condition | Treatment employed | Response |
|
| Mayer et al | 2008 [1] | 2 | SLE (2) | (1) No treatment
(2) Prednisone (30 mg/day) and azathioprine (100 mg/day) | (1) Did not require treatment
(2) Response after unclear duration |
|
| Wondergem et al | 2006 [4] | 1 | SLE | Prednisone (1 mg/kg/day unknown duration), IVIG | Persistent hemolysis despite prednisone, response to IVIG and subsequent tapering of steroids with resolution of hemolysis |
|
| Sudha Reddy et al | 2011 [5] | 1 | Unknown | Prednisolone (2 mg/kg/day for 4 weeks), tapered to alternating day steroids | Response, tapered to corticosteroids on alternating days |
|
| Tanaka et al | 2006 [6] | 1 | Following chicken pox infection | Methylprednisone (1000 mg/day unclear duration), prednisolone (60 mg/day for 4 weeks tapered to 10 mg/daily) | Initial response to methylprednisolone, worsening thrombocytopenia following taper treated with reinitiation of methylprednisone and IVIG (400 mg/day 3 days) with stabilization, recurrent hemolysis requiring reinitiation of prednisone |
|
| Win et al | 2007 [7] | 2 | (1) Unknown
(2) Splenic T-cell angioimmunoblastic non-Hodgkin’s lymphoma | (1) Prednisone (40 mg/day unclear duration) and IVIG (0.4 mg/kg for 5 days)
(2) prednisone (60 mg/kg, increased to 100 mg/kg unclear duration) and IVIG (2 mg/kg for 2 days); VCP (cyclophosphamide 750 mg/m2, vincristine 2 mg and prednisone 50 mg unclear number of cycles); rituximab (325 mg/m2 for 2 weeks); splenectomy | (1) Response with stabilization in hemoglobin
(2) No response to steroids/IVIG, chemotherapy or rituximab; response to splenectomy with stabilization in hemoglobin |
|
| Morselli et al | 2002 [8] | 1 | Unknown | Prednisone (1 mg/kg/day) with taper; over three weeks to 25 mg/daily, rituximab (325 mg/m2 for 2 weekly courses) | Response to initial steroid treatment with following taper, recurrence in hemolysis with response and stabilization in hemoglobin with 2 cycles of rituximab |
|
| Qiao et al | 2016 [9] | 1 | Primary Sjorgen syndrome | Methylprednisone (40 mg/day unknown duration) transitioned to pulse methylprednisone (1000 mg/day for 3 days) followed by prednisone 50 mg/day (unknown duration), patient received G-CSF and cyclophosphamide 0.2 mg every other day to treat concomitant agranulocytosis | Complete response with resolution of hemolysis |
|
| Imataki et al | 2020 [10] | 1 | Idiopathic, prior autologous stem cell transplant for DLBCL | Prednisolone (1 mg/kg for unknown duration) | Progressive hemolysis without response |
|
| Hirano et al | 2016 [11] | 1 | SLE | Prednisolone (2 mg/kg for 2 months and subsequent taper), 2 courses of methylprednisone pulse (unknown dosage/duration) MMF (1000 mg/day tapered 500 mg/day for unknown duration) | Response with stabilization in hemoglobin |
|
| Scaramucci et al | 2005 [12] | 1 | Idiopathic | Prednisone (1 mg/kg/day unknown duration with taper), rituximab (375 mg/m2 four weekly courses) | Response with prednisone with relapse following taper, complete response to rituximab |
|
| Elharake et al | 2019 [13] | 1 | EBV PCR positive | Methylprednisone (unknown dosage and duration), plasma exchange | Response with hemoglobin stability |
|
| Haller et al | 2009 [14] | 1 | Post liver transplant without evidence of alloantibodies, EBV and CMV positive | IV methylprednisone (1 mg/kg QID for 7 days) transitioned to prednisolone (1 mg/kg daily), rituximab (325 mg/m2 for 4 weekly courses), IVIG (0.5 g/kg, dose for 3 weekly doses), prednisolone tapered to 2.5 mg daily over 6 months and subsequently continued as GVHD prophylaxis | No response to initial methylprednisone, response to rituximab with stabilization in hemoglobin and resolution of transfusion requirements |
|
| Zhang et al | 2012 [15] | 1 | Hodgkin lymphoma | IV methylprednisone (unclear dose and duration), 1 cycle of rituximab (unclear dose) | No response to initial methylprednisone, response to rituximab with hemoglobin stabilization |
|
| Rai et al | 2017 [16] | 2 | Idiopathic | Both cases received corticosteroids (unknown medication, dose and duration) | Both had response to corticosteroid therapy with improvement in hemolysis however long term follow up unknown |
|
| Gupta et al | 2011 [17] | 1 | Idiopathic | IV methylprednisone (unknown dose and duration), plasmapheresis for 7 daily doses, rituximab (375 mg/m2 for four weekly doses) | No response to initial corticosteroid or plasmapheresis, stabilization in hemoglobin following initiation of rituximab and transfusion independence |
|
| Webster et al | 2004 [18] | 1 | Idiopathic | Cyclophosphamide 50 mg daily and prednisone 10 mg alternating days for 1 month; rituximab 700 mg IV for 4 weekly doses | Discontinued cyclophosphamide/prednisone due to side effects and progressive hemolysis, response to rituximab with resolution of transfusion requirements and hemoglobin stabilization |
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