Objective:

We evaluated the clinical features and the effects of various treatment modalities on the clinical course in patients diagnosed with immune thrombocytopenia (IT)

Patients and methods:

Retrospective investigation of the medical records of consecutive patients with IT at our center between January 2010 and December 2016 was done. Complete response (CR) was defined as any platelet count >50,000/mm³ lasting for 3 months or longer without treatment.

Results:

Of the 113 patients, 95 (84.1%) were women. At initial diagnosis, the median age of the patients was 42.5 ± 18.5 years [range : 17-82]. At the admission, 107 (94.7%) had signs of bleeding. Platelet counts were median at 9058/mm³ [1000-45000] and 88 (77.9%) patients had thrombocytopenia<10.000/mm³. The average hemoglobin level was 11.6 ± 2 g/dL [4-17] and 78 patients (69%) had anemia (Hb < 12 g/dL). In univariate analysis, hemoglobin (p <0.001) and platelet counts were significantly lower in females (p = 0.002). The hemorrhagic score was significantly higher in subjects older than 65 years (p = 0.002)

Three patients had antinuclear antibodies and one patient had an IgG type anticardiolipin. All patients had a negative serology for hepatitis C, B and HIV. Bone marrow study with aspiration showed peripheral thrombocytopenia in all cases. Erythroblastopenia was associated in one case and myelodysplastic syndrome was associated in one case. Nine patients had a history of type 2 diabetes, and two patients had a history of thyroiditis.

Follow-up was complete in all 113 patients and the median follow-up was 24.8 months (range: 6-84) with no death reported. Initial treatment with either standard or high-dose steroid as first-line therapy was begun in all patients. Pulse of methyl prednisolone was prescribed in 103 (91%) patients and immunoglobulin (Ig IV) in 7 (8%) patients. A first CR was achieved in 97% of the patients. In 3 refractory patients, a splenectomy was performed in 2 patients because of a non response and an active hemorrhage, and rituximab in one patient.

With a median delay of 21 months [3-120], hematologic relapse was noted in 62 (55%). A second relapse was noted in 10 patients (8.4%) patients. Splenectomy was performed in 22 patients (after first or second relapse) and CR was obtained in 77%. Rituximab (at different doses) was used in 19 patients at the first or second relapse. CR was obtained in 13 (68%).

For the other patients, the therapeutic alternatives were azathioprin in 3 cases, cyclophosphamide in 3 cases and danatrol in one case and eltrombopag in one case.

Conclusion: In our study, female sex was dominant and the failure of early therapeutic response is high in our series. The presentation is severe with a high hemorrhage score. Steroid therapy is effective both in the initial and relapse periods. Splenectomy remain for us the treatment of choice for those IT patients refractory to steroid therapy.

Disclosures

No relevant conflicts of interest to declare.

Author notes

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Asterisk with author names denotes non-ASH members.

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