J Korean Med Sci.  2013 Mar;28(3):472-475. 10.3346/jkms.2013.28.3.472.

Two Cases of Refractory Thrombocytopenia in Systemic Lupus Erythematosus that Responded to Intravenous Low-Dose Cyclophosphamide

• Park HJ
• Kang MI
• Kang Y
• Chung SJ
• Lee SW
• Park YB
• Lee SK

• KMID: 1786948
• DOI: http://doi.org/10.3346/jkms.2013.28.3.472

Abstract

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/microliter. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/microliter after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.

Keyword

Cyclophosphamide; Systemic Lupus Erythematosus; Thrombocytopenia

MeSH Terms

Azathioprine/therapeutic use
Bone Marrow/pathology
Cyclophosphamide/*therapeutic use
Drug Therapy, Combination
Female
Humans
Immunosuppressive Agents/*therapeutic use
Infusions, Intravenous
Lupus Erythematosus, Systemic/complications/*diagnosis
Middle Aged
Mycophenolic Acid/analogs & derivatives/therapeutic use
Platelet Count
Thrombocytopenia/*diagnosis/*drug therapy/etiology
Young Adult
Immunosuppressive Agents
Mycophenolic Acid
Azathioprine
Cyclophosphamide

Figure

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  Fig. 1 Changes of platelet count in patients with multiple medical treatments. (A) In case 1, the pateint recurred severe thrombocytopenia and recieved methylpredinoslone pulse therapy for 3 days and intravenous immunoglobulin (IVIG) for 5 days. She did not respond these medications and started low-dose intravenous cyclophosphamide (IV CYC) therapy. She achieved remission of thrombocytopenia after the first IV CYC therapy, and a stable platelet count was maintained. (B) In case 2, the platelet count of patient was decreased below 10,000/µL and received methylpredinoslone pulse therapy and IVIG. However, this combination treatment showed no efficacy. The platelet count was increased above 50,000/µL after three cycles of IV CYC, and it remained at stable level. Doses of steroids are presented according to the potency of prednisolone. MP pulse therapy, intravenous methylprednisolone pulse therapy (1,000 mg/day for 3 days); IVIG, intravenous immunoglobulin; IV CYC, intravenous infusion of low-dose cyclophosphamide.

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