Figure 3.
Classification of drugs used for the treatment of immune-mediated cytopenia according to their mechanism of action, toxicity, target, or symptom specificity and treatment costs. The large overlap of some of these subgroups (especially of anti-inflammatory drugs, immunosuppressive drugs, “targeted” drugs) is not shown for clarity. 6-MP, 6-mercaptopurine; anti-D, anti-Rh(D) antibody; AZT, azathioprine; CSA, cyclosporin A; CY, cyclophosphamide; IVIG, high-dose intravenous immunoglobulin G; JAK-inhibs, inhibitors of Janus kinases; MMF, mycophenolate mofetil; MTX, methotrexate; TNFa, tumor necrosis factor α; TRA, thrombopoietin receptor agonists; VCR/VBL, vinca alkaloids. 1Rarely used; etoposide in hemophagocytic lymphohistiocytosis; 2use in strictly defined indications; 3use based on largely anecdotal evidence, ideally under clinical trial conditions or compassionate use with informed consent; 4ibrutinib (targeting BTK, ITK), belimumab (anti-BAFF), epratuzumab (anti-CD22), carfilzomib (proteasome inhibitor); 5G-CSF in severe aplastic anemia, CXCR4 inhibition in myelokathexis, rarely erythropoietin analogs; 6the overall toxicity is difficult to measure, taking teratogenicity, myelo- and organ toxicity, immunosuppression, reversibility, and procedural risks into account, and is schematically illustrated here without considering individual risk factors (intolerance and acquired or inherited risk factors, eg, for thromboembolism or allergic reactions) or potential specific adverse effects; $-$$$a very rough estimate of procurement and treatment costs over repeated or continuous application is presented from “budget” ($, ≤US$1500/year) to high ($$$, ≥US$15 000-20 000/year).

Classification of drugs used for the treatment of immune-mediated cytopenia according to their mechanism of action, toxicity, target, or symptom specificity and treatment costs. The large overlap of some of these subgroups (especially of anti-inflammatory drugs, immunosuppressive drugs, “targeted” drugs) is not shown for clarity. 6-MP, 6-mercaptopurine; anti-D, anti-Rh(D) antibody; AZT, azathioprine; CSA, cyclosporin A; CY, cyclophosphamide; IVIG, high-dose intravenous immunoglobulin G; JAK-inhibs, inhibitors of Janus kinases; MMF, mycophenolate mofetil; MTX, methotrexate; TNFa, tumor necrosis factor α; TRA, thrombopoietin receptor agonists; VCR/VBL, vinca alkaloids. 1Rarely used; etoposide in hemophagocytic lymphohistiocytosis; 2use in strictly defined indications; 3use based on largely anecdotal evidence, ideally under clinical trial conditions or compassionate use with informed consent; 4ibrutinib (targeting BTK, ITK), belimumab (anti-BAFF), epratuzumab (anti-CD22), carfilzomib (proteasome inhibitor); 5G-CSF in severe aplastic anemia, CXCR4 inhibition in myelokathexis, rarely erythropoietin analogs; 6the overall toxicity is difficult to measure, taking teratogenicity, myelo- and organ toxicity, immunosuppression, reversibility, and procedural risks into account, and is schematically illustrated here without considering individual risk factors (intolerance and acquired or inherited risk factors, eg, for thromboembolism or allergic reactions) or potential specific adverse effects; $-$$$a very rough estimate of procurement and treatment costs over repeated or continuous application is presented from “budget” ($, ≤US$1500/year) to high ($$$, ≥US$15 000-20 000/year).

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