Table 2.
When we look at the usage in patients which have a primary diagnosis
before getting IVIG therapy; out of 87 patients (52.4%), 25 patients
were diagnosed with ITP (28.7%), 7 patients with KLL (19.5%), 1 with
autoimmune hemolytic anemia (1.1%), 1 with myelodysplastic syndrome
(1.1% ), 3 CVID (3.4%), 4 with AML (4.6%), 1 (1.1%), 4 with Hodgkin
lymphoma (4.6%), 7 with non-Hodgkin lymphoma (8%), 14 with multiple
myeloma, 1 with Waldenström macroglobulinemia (1.1%), 1 with hairy cell
leukemia (1.1%), 2 with PRCA (2.3%), 2 with AIDS (2.3%), 1with SLE
(1.1%), 1 with Castleman Disease (1.1%) and 2 was diagnosed with
sickle cell anemia (2.3%). Besides primary disease, the indications for
IVIG use are given in Table 3. Immun thrombocytopenia is
targeted in all cases diagnosed with ITP. It was used in the treatment
of immune thrombocytopenia (23.5%) in 4 of 17 patients with CLL,
secondary hypogammaglobulinemia in 11 (64.7%) and autoimmune hemolytic
anemia in 2 (11.8%). It can be said that these rates are in line with
the literature. Although different figures are mentioned in various
publications, CLL-related autoimmune-origin cytopenia is reported on
average around 20-25%. (6) In MDS, AML, ALL, AIDS and Waldenström
patients with thrombocytopenia, IVIGs have been used off-label with the
approval of the ministry of health in the treatment of secondary to
immune-origin, resistant or alloimmunization. It has been observed that
5 (8%) of the 7 patients diagnosed with non-Hodgkin lymphoma have been
administered IVIG therapy; it was used for the treatment of
immune-induced thrombocytopenia in 5 (71.4%) and in 2 (28.6%) for the
treatment of secondary hypogammaglobulinemia and secondary immune
deficiency.
Of the 14 patients (16.1%) diagnosed with multiple myeloma, 5 (35.7%)
had IVIG therapy for the treatment of alloimmune thrombocytopenia and 9
(64.3%) for secondary hypogammaglobulinemia. Table 3.
The patients and their distributions were evaluated according to the
targets determined for the response. The expected response was 36.1%
with 60 patients within the entire patient group. In 108 patients, the
target response could not be obtained (63.9%). In the ITP patient
group, the response was 56.1% out of a total of 37 patients; 8 patients
in CLL (42.1%), 1 patient in autoimmune hemolytic anemia (100%), 3
patients in CVID (100%), 1 patient in ALL (12.5%), 1 patient in HL
(25%), It resulted in 5 patients (35.7%) in MM, 1 patient in HCL
(100%), 2 patients in PRCA (100%), 1 patient in AIDS (33.3%). In
patients with primary diagnosis of MDS, AA, AML, NHL, WM, sickle cell
anemia and Castleman Disease, the target response could not be obtained
with various indications. Table 4.
The response was 58.5% with 24 patients in patients who were not
diagnosed with any disease subgroup before getting IVIGs , were
diagnosed with ITP with urgent and deep thrombocytopenia after IVIG
usage . No response was obtained in other patients. Table 5.
In patients who were diagnosed with a primary disease prior to the usage
of IVIGs; considering the various indications, usage and responses
mentioned previously: 52% with 13 patients in ITP, 47.1% with 8
patients in CLL, 100% with 3 patients in CVID, and 100% with 1 patient
in ALL , 25% with 1 patient in Hodgkin Lymphoma, 35.7% with 5 patients
in multiple myeloma, 100% with 1 patient in Waldenström
Macroglobulinemia and HCL, 50% with 1 patient in PRCA, sickle cell
anemia and AIDS. Table 6.
Table 7 shows the patients used for prophylaxis. The
distribution of patients who are preferred for the treatment of
secondary hypogammaglobulinemia and secondary immunodeficiency can be
seen. Out of 21 patients, 6 were diagnosed with multiple myeloma
(28.6%), 6 with KLL (28.6%), 3 with non-Hodgkin lymphoma (14.3%), 1
with Hodgkin Lymphoma (4.8%), 3 with CVID (% 14.3) and 2 of them were
diagnosed with sickle cell anemia (9.5%). Two patients with sickle cell
anemia were otosplenectomized; it should be noted that they received
treatment with the indication of secondary hypogammaglobulinemia.