Table 3.

The use of intravenous immunoglobulins in hematological diseases

Disease	Ref.	Study design	Intervention including dose and IVIg preparation used	Number of patients	Results/response
Acquired hemophilia	[92]	Prospective multicenter study	Induction with IVIg (5% Gamimune-N, Miles Inc, Berkeley, CA, USA) 2 g/kg over 2 or 5 days, maintenance with 0.4 g/kg by clinician decision	19 patients with acquired factor VIII inhibitors	8 of 16 assessable patients (50%) had inhibitors reduced in more than 25%; in one third of these responses, concomitant CS treatment may have influenced results
	[93]	Review of published case reports	Different IVIg preparations, dose ranging from 1.6 to 2.8 g/kg over 2–7 days	26 patients with acquired factor VIII inhibitors	16 of 26 patients (62%) had inhibitors reduced in more than 25%; 7 of 26 patients (27%) had clinical benefit
	[93]	Case series	IVIg (Sandoglobulin) 2 g/kg over 5 days	4 patients with acquired factor VIII inhibitors	2 of 4 patients (50%) had inhibitors reduced in more than 25%, but with no clinical benefit; in 2 of 4 patients, the inhibitor reductions were no assessable due to concomitant CS or CP treatment, with clinical benefit in doubt
	[94]	Review of published case reports	Different IVIg preparations and doses; IVIg therapy alone	35 patients with acquired factor VIII inhibitors	30% had inhibitors reduced in more than 25% and clinical benefit
	[95]	Case series	Prednisolone 1 mg/kg per day with IVIg 2 g/kg over 2–5 days	7 patients with acquired factor VIII inhibitors	5 of 7 patients (71%) had inhibitors reduced in more than 25%
Acquired hypogammaglobulinemia	[96]	RCT crossover study	6 months IVIg (0.3 g/kg per month, Ig-Vena N, Sclavo, Siena, Italy) or placebo, then switch for 12 months and switch again for 6 months	42 CLL patients with hypogammaglobulinemia	IVIg treatment yielded more infection free patients at 6 months than placebo (20 vs. 9, p < 0.01) as well as at 12 months (13 vs. 6, p < 0.02)
	[97]	RCT crossover study	12 months IVIg 0.4 g/kg (Gammagard, Hyland Therapeutic Division, Baxter Healthcare, Glenoak, CA, USA) every 3 weeks or placebo, then switch for 12 months	12 B cell CLL or NHL patients with hypogammaglobulinemia	During IVIg treatment, there were more infection-free patients than during placebo administration (6 vs. 1, p = 0.001); in addition, 10 of 12 had lower rates of severe bacterial infection while on IVIg (p = 0.001)
	[98]	RCT	18 g IVIg or albumin each 3 weeks for 12 months	42 CLL patients with hypogammaglobulinemia	Fewer infections in IVIg-treated (29% vs. 61%, p = 0.04)
					Fewer severe infections in IVIg-treated (21% vs. 56%, p = 0.02)
	[99]	RCT	IVIg 0.4 g/kg (Gammagard, Baxter Healthcare) or albumin every month for 12 months	82 patients with multiple myeloma	19 serious infection in IVIg-treated compared with 38 in placebo-treated patients (p = 0.019)
					No septicemia/pneumonia on IVIg treatment compared with 10 cases in placebo-treated (p = 0.002)
	[100]	RCT	IVIg (Endobulin, Immuno) 0.4 g/kg or no treatment each month, for 6 months	60 children with acute lymphoblastic leukemia	Less infections in IVIg-treated patients
	[101]	RCT	Cefataxim and amikacin with or without IVIg	33 children with acute lymphoblastic leukemia and neutropenic fever	Shorter fever duration in IVIg group (5.2 vs. 7.9 days, p < 0.05)
					Similar neutropenia and hospitalization duration
	[102]	RCT	IVIg (Gammagard Baxter Healthcare Corporation Hyland Division) 0.4 g/kg or placebo every 3 weeks for 12 months	84 CLL patients with hypogammaglobulinemia or a history of infection	Overall fewer bacterial infections in the IVIg group (23 vs. 42, p = 0.01)
					Even more marked difference in those who completed 1 year (14 vs. 36, p = 0.001)
	[103]	RCT crossover study	6 months IVIg 0.3 g/kg per month (Ig-Vena N, Sclavo, Siena, Italy) or no therapy, then switch for 12 months and switch again for 6 months	25 multiple myeloma patients with hypogammaglobulinemia	During IVIg treatment, ten serious infections occurred; during no therapy, phase 30 serious infections occurred, p < 0.002
	[104]	Two trials: RCT and RCT crossover study	12 months IVIg 0.4 g/kg every 3 weeks (Gammagard Baxter Healthcare Corporation Hyland Division) or placebo, then switch for 12 months (only in the case of the RCT crossover trial)	RCT trial: 81 patients with B cell CLL or low-grade NHL patients with hypogammaglobulinemia	RCT trial: less bacterial infections in the IVIg group (23 vs 42, p = 0.01)
				RCT crossover study: 12 patients with B cell CLL or low-grade NHL patients with hypogammaglobulinemia	RCT crossover trial: larger number of patients free of severe infections in the IVIg group (6 vs 1, p = 0.001)
Pure red cell aplasia	[105]	Case series	IVIg 2 g/kg over 5 days	3 pure red cell aplasia patients	Good response in all patients
	[106]	Case series	IVIg 2 g/kg (2 patients received Sandoglobulin, Sandoz, Switzerland, 2 patients received Veinoglobulin, Institut Merieux, France) over 5 days	4 patients with red cell aplasia, one idiopathic, another with well-differentiated lymphoma and 2 with B cell CLL	Good response in all patients
	[107]	Case report	IVIg	42-year-old male with parvovirus-B19-induced red cell aplasia after stem cell transplantation	Good response
	[108]	Case report	IVIg 2 g/kg over 5 days	38 year-old male with parvovirus-B19-induced red cell aplasia after liver transplantation	Good response
	[109]	Case report	IVIg (Panglobulin, ZLB Bioplasma, AG, Bern, Switzerland) 2 g/kg over 2 days	14-year-old male with parvovirus-B19-induced red cell aplasia after renal transplantation	Good response but development of osmotic sucrose-related renal failure
	[110]	Case report	IVIg 1 g/kg single dose	26-year-old female with parvovirus-B19-induced red cell aplasia after CHOP chemotherapy, rituximab, and radiotherapy	Good response
	[111]	Case report	IVIg	Relapsing pure red cell aplasia in a patient with B-cell chronic lymphocytic leukemia, refractory to PD and CP	Good response
	[112]	Case report	IVIg 4 g/kg over 10 days	Patient with parvovirus-B19-induced red cell aplasia after renal transplantation	Good response
	[113]	Case report	IVIg	41-year-old male patient with acquired immunodeficiency syndrome and parvovirus-B19-induced red cell aplasia	Good response
	[114]	Case series	IVIg 1 to 2 g/kg over 1 to 2 days + maintenance dose 0.4 g/kg each month	8 patients with acquired immunodeficiency syndrome and parvovirus-B19-induced red cell aplasia	Good response in 8 of 8 patients, 6 of 8 needed also maintenance IVIg
	[115]	Case report	0.3 g/kg IVIg (Venilon, Teijin, Osaka) over 6 days	28-year-old female patient with common variable immunodeficiency and parvovirus-B19-induced red cell aplasia	Good response
	[116]	Case report	1 g/kg IVIg	34-year-old male with acquired immunodeficiency syndrome and parvovirus-B19-induced red cell aplasia	Good response
	[117]	Case report	2 g/kg IVIg (Intragam, CSL) over 5 days	Male patient with acquired immunodeficiency syndrome and parvovirus-B19-induced red cell aplasia	Good response
	[118]	Case report	2 g/kg IVIg then variable maintenance doses	26-year-old male patient with acquired immunodeficiency syndrome and parvovirus-B19-induced red cell aplasia	Good response
	[119]	Case report	IVIg	Female patient with chronic idiopathic pure red cell aplasia	Good response including 2 uneventful pregnancies during treatment
	[120]	Case report	2 g/kg IVIg over 5 days	22-year-old female patient with systemic erythematosus lupus and pure red cell aplasia, after failure of CS therapy	Good and immediate response
	[121]	Case report	4 g/kg IVIg (Sandoglobulin) over 10 days, subsequent additional shorter courses and plasmapheresis to due to immune-complex disease	24-year-old female patient with parvovirus-B19-induced red cell aplasia after liver transplantation	Late response; it is questionable that the late response is due to IVIg due to the late timing
	[122]	Case report	2 g/kg IVIg (Sandoglobulin, Sandoz, East Hanover, NJ, USA) over 5 days, subsequent maintenance courses	4-year-old patient pure red cell aplasia	Good and long-term response, weaned of blood transfusions
	[123]	Case report	2 g/kg IVIg over 5 days, subsequent maintenance courses	65-year-old Waldenström's macroglobulinemia patient with parvovirus-B19-induced pure red cell aplasia	Good and long-term response, weaned of blood transfusions
Acquired von Willebrand syndrome	[7]	Prospective noncontrolled study with sequential treatments	Desmopressin and factor VIII/von Willebrand factor concentrate where compared to IVIg 2 g/kg over 2 days (Sandoglobulin, Novartis or Ig-Vena Sclavo)	10 MGUS patients with acquired von Willebrand syndrome: 8 had IgG-kappa or lambda, 2 had IgM-kappa	Transient increase in plasma von Willebrand factor with desmopressin and factor VIII/von Willebrand factor concentrate; IVIg infusion in IgG-MGUS resulted in a more sustained increase starting on day 4 and returning to preinfusion levels on day 21
	[94]	Case series	IVIg	9 acquired von Willebrand syndrome patients	Good response in 30%
	[124]	Case report	IVIg 0.9 g/kg over 3 days	43-year-old patient with multiple myeloma and gastrointestinal bleeding due to acquired von Willebrand syndrome not responsive to vasopressin	Rapid hematological correction after 4 days, bleeding control
	[125]	Case report	IVIg	2 SLE patients with von Willebrand syndrome	Good response in one of the 2 patients
Aplastic anemia	[1]	Case report	IVIg	2 patients with idiopathic aplastic anemia	No response
	[126]	Case report	IVIg	Aplastic anemia patient unresponsive to antithymocyte globulin and CS	Good response
	[127]	Case report	IVIg	Aplastic anemia due to parvovirus B19 infection in a heart transplant recipient	Good response
	[128]	Case report	IVIg (ISIVEN, Instituto Sierovaccinogeno, Italiano ISI SpA) 2 g/kg over 5 days	66-year-old woman with idiopathic aplastic anemia, unresponsive to CS	Good response
Autoimmune hemolytic anemia	[129]	Multicenter noncontrolled study + review of reported cases	IVIg (generally Sandoglobulin, Basel, Switzerland, but also Gamimmune N, Cutter Biological, Emeryville, CA, USA; Venilon, Teijin Institute, Tokyo; Sanglopor, Sankyo Institute, Tokyo) 2.5–7 g/kg over 5–7 days	73 patients with autoimmune hemolytic anemia: 36 from multicenter study, 37 from review of published cases	29 patients (39.7%) responded to IVIg: hepatomegaly and low pretreatment hemoglobin were correlated with good response
Autoimmune neutropenia	[130]	Case series	IVIg 0.4–1 g/kg for 5–3 days	20 infants with neutropenia	50% responded after IVIg as compared to 100% with G-CSF and 57% with CS
	[131]	Case series	IVIg 3 g/kg over 3 days	6 infants with neutropenia	Fast but transient rise in neutrophils in all
Evans' Syndrome	[132]	Case series	IVIg single or multiple courses + CS	40 patients (children and young adults)	Remission in 9/40, transient response in 26/40 (overall improvement in 87%)
	[133]	Case series	IVIg plus CS for either acute hemolysis or thrombocytopenia	5 pediatric patients	Transient effect that needed immunosuppressant therapy for maintenance
	[134]	Case report	IVIg 0.4 g/kg for 5 days	3 patients with ES refractory to conventional therapy, including CS and splenectomy in all of the patients, vincristine in 2, and CP in one	2 patients failed to respond, but the third had a clinical remission after IVIg therapy
	[135]	Case report	IVIg 0.4 g/kg for 5 days	A patient with steroid resistant ES associated with dermatomyositis	IVIg was transiently effective, but a sustain remission was achieved with CP
Fetal/neonatal alloimmune thrombocytopenia	[136]	Nonrandomized study	IVIg, 1 g/kg weekly, or CS given to pregnant women	37 pregnant women with previous pregnancy with alloimmune thrombocytopenia: 27 received IVIg, 10 received CS	There was an increase in platelets and no intracranial bleeding in 26% of patients/fetus treated with IVIg as compared with 10% of those treated with CS; in addition, there was no increase in platelets and no intracranial bleeding in 41% of patients/fetus treated with IVIg as compared with 20% of those treated with CS
Stem cell/bone marrow transplantation: infections and graft-versus-host disease	[137]	RCT	16 weekly doses since 1 week before transplantation of either IVIg (Sandoglobulin, Novartis Pharma, Rueil-Malmaison) 0.05, 0.25, and 0.5 g/kg or placebo	200 patients with allogeneic stem cell transplantation	No protection against infections; GVHD and mortality were similar
	[138]	Randomized trial	64 patients received 0.4 g/kg per week of IVIg (Sandoglobulin, Sandoz, Switzerland); 64 patients received 0.1 g/kg per week of CMV-IgG	128 patients with allogeneic bone marrow transplantation	No significant difference in the occurrence or severity of acute or chronic GVHD, infections, or survival
	[139]	RCT	123 patients received 0.5 g/kg per month of IVIg; 127 patients did not receive IVIg, from the fourth to the 12th month after transplantation	250 patients with allogeneic bone marrow transplantation	No significant difference in the occurrence of chronic GVHD, infections, or survival
	[140]	Randomized trial	All received CMV-negative blood products, 25 received IVIg (1 g/kg per week) starting before pretransplant conditioning and then for 17 additional weeks	48 patients after allogeneic BMT, CMV seronegative, which received CMV seropositive or seronegative BMT	No difference in the number of bacterial/fungal infections, fewer non-CMV viral infections in the IVIg group (9 vs 15, p = 0.03), less grade ≥II of GVHD in the IVIg group, p = 0.04
	[141]	Multicenter RCT	IVIg (Venoglobulin S 5%, Alpha Therapeutic Corporation, LA, CA, USA), 0.1, 0.25, or 0.5 g/kg, started 2 days before transplant, continued weekly for 90 days and then monthly until 1 year after transplant	618 allogeneic bone marrow transplant recipients	Acute GVHD occurred in 39% (80/206) in the 0.1-g/kg group, 42% (88/208) in the 0.25-g/kg group, 35% (72/204) in the 0.5 g/kg group
					No difference in incidence of chronic GVHD, infection, interstitial pneumonia, type of infection, relapse of hematological malignancy or survival
	[142]	Randomized trial	IVIg (Sandoglobulin, Sandoz Pharmaceuticals, East Hanover, NJ, USA) 0.5 g/kg per week administered since the beginning of the cytotoxic therapy or nothing	170 autologous bone marrow transplant patients	IVIg did not reduce infection of infection-related death
	[143]	RCT	IVIg (Gamimune N, Cutter Biological, Berkeley, CA, USA) 0.5 g/kg per week to day 90, then 0.5 g/kg per month to day 360 after transplantation or nothing	382 patients after bone marrow transplantation	Lower risk of gram-negative septicemia (p = 0.0039) in IVIg and of local infection (p = 0.029)
					No difference in survival; there was a risk reduction in the incidence of acute GVHD (p = 0.0051)
					Decrease in deaths due to transplant-related causes after transplantation of HLA-identical marrow (p = 0.023)
Hemolytic disease of the newborn	[144]	Meta-analysis of 3 not-blinded RCT	IVIg and phototherapy or phototherapy alone	189 patients	Less use of exchange transfusion in the IVIg group: when given for prophylaxis RR = 0.21 (p < 0.001), when given as treatment RR = 0.36 (p < 0.006)
	[145]	Meta-analysis of 3 RCTs	IVIg and phototherapy or phototherapy alone	189 patients	Overall less use of exchange transfusion in the IVIg group: RR = 0.28 (p < 0.00001)
Hemolytic transfusion reaction	[146]	Case series	IVIg (Endobulin HT, Immuno AG, Vienna, Austria) 0.4 g/kg (single infusion) was administered within 24 h of transfusion	5 patients are known to develop non-ABO post-transfusional hemolytic reactions	No transfusion reaction and sustained increase in hematocrit
	[147]	Case report	IVIg (Sandoglobulin) 75 gr. over 3 days + CS	Rh-negative patient with anti-Jk, anti-K, and anti-Kp	Increase in hemoglobin, no evidence for hemolysis
Hemolytic transfusion reaction in sickle cell disease	[148]	Case reports	IVIg 2 g/kg over 5 days + high-dose MP	2 patients with sickle cell disease who developed a hemolytic reaction after compatible blood transfusion	Increase in hemoglobin and reticulocytes
	[149]	Case reports	IVIg 2 g/kg over 5 days + high-dose MP	2 patients with sickle cell disease who developed a hemolytic reaction after compatible blood transfusion	Increase in hemoglobin and reticulocytes
	[150]	Case report	IVIg (Sandoglobulin, 1 g/kg per day for 1 to 2 days) + CS	Patient with sickle cell disease who developed a hemolytic reaction after compatible blood transfusion	Increase in hemoglobin and reticulocytes
Hemolytic uremic syndrome	[151]	Case control study	IVIg 0.4–2.4 g/kg (Gamimune N, Cutter Etobicoke, Ontario, Canada) over 1–6 days (0.4 g/kg per day)	18 children with postdiarrhea hemolytic uremic syndrome, 9 received IVIg	No benefit of IVIg treatment
	[152]	Case control study	IVIg 2 g/kg (Sandoglobulin) over 5 days in 8 patients, FFP in 12 patients, no treatment in 23 patients	43 children with hemolytic uremic syndrome; 8 received IVIg	Improvement in platelet count against FFP (p < 0.05) and against no treatment (p < 0.01)
Thrombotic thrombocytopenic purpura	[153]	Case series	IVIg 0.4 g/kg per day, from 1 to 20 infusions; these patients were also treated with CS, also PP in 15 patients	17 patients with thrombotic thrombocytopenic purpura	10/17 (58.8%) patients had remission, 8/17 (47%) complete remission
	[154]	Case control study	IVIg 2 g/kg over 5 days in 29 patients only; all the patients received PP and CS	44 patients with thrombotic thrombocytopenic purpura	No benefit of IVIg treatment
Heparin-induced thrombocytopenia	[155]	Case series	IVIg (Sandoglobulin in 2 cases, Octagam in one case) 2 g/kg over 2 days	3 patients with heparin-induced thrombocytopenia	Increase in platelet count
	[156]	Case report	IVIg	51-year-old woman with heparin-induced thrombocytopenia and pulmonary embolism	Increase in platelet count starting 20 h after first IVIg infusion
HIV-associated thrombocytopenia	[157]	RCT crossover study	Weekly IVIg (Polygam) courses of 2 g/kg over 2 days or normal saline, for 4 weeks, then crossover	12 HIV patients with HIV-related thrombocytopenia	IVIg consistently and reproducibly raised platelet count after infusion; no patient with placebo did so (p < 0.00003)
Acute immune thrombocytopenic purpura	[158]	RCT not blinded	IVIg 2.1 g/kg over 3 days (Gammagard SD, Baxter Bioscience, Glendale, CA) or high-dose MP 15 mg/kg per day for 3 days	Acute immune thrombocytopenic purpura adult patients, 56 randomized to IVIg, 60 to MP	Faster response and higher platelet counts in the IVIg group (p = 0.006)
	[159]	RCT nonblinded	IVIg 2 g/kg over 5 days (7 patients), PD 1 mg/kg per day (13 patients) or both IVIg and PD (12 patients)	32 acute immune thrombocytopenic purpura adult patients	No difference in response rates or in requirements for splenectomy or in bleeding
	[160]	RCT nonblinded	IVIg 2 g/kg over 2 days (19 patients) or PD with starting dose of 4 mg/kg per day and then tapering until discontinuation by day 21 (18 patients) or no therapy (16 patients)	53 children with acute immune thrombocytopenic purpura and less than 20,000 platelets per microliter	Both IVIg- and PD-treated children reached platelet counts over 50,000 per microliter faster than children not treated (p < 0.001)
					Median time to reach more than 50,000 platelets per microliter was lower in IVIg group (2 days) than in PD group (4 days), p < 0.001
	[161]	Partially randomized trial; patient's family chose no treatment or treatment (IVIg or CS upon randomization)	IVIg 1.6 g/kg over 2 days (12 patients) or MP with starting dose of 30 mg/kg per day for 3 days and then 20 mg/kg per day for 4 days (12 patients) or no therapy (26 patients)	50 children with acute immune thrombocytopenic purpura and less than 20,000 platelets per microliter	Both IVIg- and MP-treated children reached platelet counts over 20,000 per microliter and 50,000 per microliter faster than children not treated, p < 0.01
Immune thrombocytopenic purpura in HIV	[162]	Controlled clinical trial	IVIg 2 g/kg of IVIg over 2 days, followed by IVIg 1 g/kg on day 15	14 patients with HIV-related thrombocytopenia (median platelet count 17,000/mm3)	All achieved resolution of their bleeding by day 8, but this was temporary
	[163]	Controlled clinical trial	IVIg 0.04 g/kg per week during 5 weeks	13 thrombocytopenic AIDS patients	All patients responded in the first week but only 4 were responders after 3 months
Chronic immune thrombocytopenic purpura	[164]	Prospective noncontrolled trial	IVIg 2 g/kg over 2 or 5 days (BT681m, Biotest Pharma GmbH, Dreieich, Germany) in accordance to randomization, followed for 28 days	24 chronic immune thrombocytopenic purpura adult patients	91.7% of patients underwent a fast raise (in 2–5 days) of platelet count over 50,000 per microliter; at the end of the 28 days of follow-up, half of the patients had still platelet count over 50,000 per microliter
	[165]	Prospective noncontrolled trial	Induction with IVIg 1 or 2 g/kg (Biotransfusion, Roissy, France) over 2 days and 6 more maintenance infusions of IVIg 1 g/kg starting when platelets fell below 50,000 per microliter and then every 2 to 3 weeks unless platelets are over 150,000 per microliter or response is exhausted	20 chronic immune thrombocytopenic purpura adult patients	Initial response in all 18 evaluable subjects, 13 complete (>150,000 per microliter), 5 partial (>50,000 per microliter); no difference between both induction doses; maintenance: at 90-day failure in 61% (11 of 18), partial response (>50,000 per microliter in 2 of 18, 11%) and complete response (>150,000 per microliter in 5 of 18, 28%)
Posttransfusional purpura	[166]	Case series	IVIg	17 patients with severe thrombocytopenia after blood transfusion	In 16 patients, normal platelet counts were reached within days; 5 patients relapsed and again had a good response to new IVIg administration
	[167]	Case series	IVIg 0.4 g/kg per day, for 2–10 days	5 patients with severe thrombocytopenia after blood transfusion	Immediate raise in platelet count and cessation of bleeding in 4 of 5 patients (80%)
	[168]	Case series	IVIg, variable doses ranging from 1 to 2 g/kg per day	3 patients with thrombocytopenia after blood transfusion	Good response in 2 of 3 patients
Hemophagocytic syndrome	[169]	Case series	IVIg	6 renal transplant patients with hemophagocytic syndrome	Good response in all the cases
	[170]	Case series	IVIg	7 children with hemophagocytic syndrome treated with IVIg only	3 of 7 (43%) survived
	[171]	Case series	IVIg 2 g/kg over 2 days in one case, over 5 days in the other	2 cases of EBV-associated hemophagocytic syndrome treated with IVIg only	2 of 2 (100%) survived
	[172]	Case series	IVIg	8 children with infection-associated hemophagocytic syndrome treated with IVIg	0 of 8 (0%) survived
	[173]	Case series	IVIg 1 g/kg per day for 1 or 2 days	3 children with infection-associated hemophagocytic syndrome treated with IVIg	3 of 3 (100%) survived
	[174]	Case report	IVIg	A healthy patient with CMV-related hemophagocytosis	Symptoms and laboratory abnormalities improved dramatically after the onset of the treatment
POEMS syndrome	[175]	Case report	2 courses of IVIg 0.4 g/kg per day for 5 days, separated by 3 weeks, adjunctive to radiotherapy	49-year-old patient with rapidly progressive polyneuropathy associated with osteosclerotic myeloma	After 2 courses of IVIg improvement of respiratory and sexual function and gate, disappearance of numbness; doing well after 1 year
	[176]	Case report	IVIg 0.4 g/kg per day for 4 (case 1) or 5 days (case 2) adjunctive to prednisolone 30–50 mg/day	A 55-year-old man (case 1) and a 43-year-old woman (case 2) with late-stage POEMS	No change in motor and sensory impairment; case 1 continued to deteriorate and died after 6 months due to sepsis and DIC
	[177]	Case report	IVIg (one course)	A patient with POEM and Castleman's disease	No effect
Diffuse B cell posttransplant lymphoproliferative disorder	[178]	Case report	IVIg 0.5 g/kg every 15 days for 4 months (case 1) and for 12 and 7 months, respectively (case 2) adjunctive with IFN alpha (2 × 106 IU SC 3 times a week)	2 patients with diffuse B cell posttransplant lymphoproliferative disorder	Complete disappearance of all lesions after 3 months of the therapy in case 1 and after 7 months in case 2, remission for 47 months and 33 months, respectively

POEMS syndrome polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes