Severe Autoimmune Thrombocytopenic Purpura Research Articles

Pregnancies Complicated by Severe Autoimmune Thrombocytopenic Purpura

Pregnancies Complicated by Severe Autoimmune Thrombocytopenic Purpura

Crohnʼs disease associated with severe autoimmune thrombocytopenic purpura

Crohnʼs disease associated with severe autoimmune thrombocytopenic purpura

Intravenous Immunoglobulin for Autoimmune Thrombocytopenic Purpura

Severe autoimmune thrombocytopenic purpura is now commonly treated with high doses of intravenous immunoglobulins (IVIGs). Twenty-four years after this treatment was first demonstrated to be effective, several questions remain to be resolved. We review here current knowledge concerning the frequency and type of side effects and the likely mechanism of action of IVIGs. We suggest that the currently recommended dose of IVIG (2 g/kg) could be halved, that the total dose of IVIG should be administered as a single infusion, that nonresponders could be provided another equal dose on day 3, and that IVIG plus prednisolone should be considered the gold standard for treatment of the most severe forms of the disease. Treatment with anti-D immunoglobulin could be proposed as an alternative if the results recently obtained with high doses (75 μg/kg) are confirmed. Finally, because IVIG has only a transient effect, it cannot be considered a curative treatment for patients with chronic autoimmune thrombocytopenic purpura.

Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial

Treatment of adults with autoimmune thrombocytopenic purpura (AITP) is based more on individual experience than on results of controlled studies. We compared intravenous immunoglobulin with high-dose methylprednisolone in untreated adults with severe AITP and assessed efficacy of subsequent oral steroids compared with placebo. Primary outcome was number of days with platelet count greater than 50 x 10(9)/L within the first 21 days. We did a randomised multicentre trial based on a 232 design. 122 adults with severe AITP (platelet count < or =20 x 10(9)/L) were randomly assigned to receive either intravenous immunoglobulin or high-dose methylprednisolone on days 1-3 (randomisation A), and then to receive either oral prednisone or placebo (randomisation B) on days 4-21. Analysis was by intention to treat. Six patients were excluded from the analysis. The number of days on which platelet counts were above 50 x 10(9)/L was 18 in 56 patients receiving intravenous immunoglobulin and 14 in 60 receiving high-dose methylprednisolone (p=0.02). Percentage of patients who had platelet counts over 50 x 10(9)/L on days 2 and 5 was 7% and 79%, respectively, in the intravenous immunoglobulin group compared with 2% and 60%, respectively, in the high-dose methylprednisolone group (p=0.04). During the second treatment period, prednisone was more effective than placebo for all short-term endpoints. Patients who received intravenous immunoglobulin and prednisone had platelet count greater than 50 x 10(9)/L for 18.5 days (p=0.005), and those treated with high-dose methylprednisolone and prednisone had this count for 17.5 days. Intravenous immunoglobulin and oral prednisone seems to be more effective than high-dose methylprednisolone and oral prednisone in adults with severe AITP, although the latter treatment is effective and well tolerated.

A randomized clinical trial comparing intravenous immunoglobulin with high-dose intravenous methylprednisolone, with or without subsequent oral prednisone administration, in adults with previously untreated severe autoimmune thrombocytopenic purpura

To compare the response to intravenous immunoglobulin (IVIg) with the response to high-dose methylprednisolone (HDMP) in previously untreated adults with severe AITP and to determine if oral prednisone (PRDN) is required after IVIg or HDMP infusions. M ethods 116 adults, previously untreated, with platelet count ≤ 20 × 109/L were assigned by simultaneous double randomization to receive either IVIg (0·7 g/kg body weight per day) or HDMP (15 mg/kg body weight per day, total daily dose less than 1 g) from day 1 to day 3 (randomization A), and then to receive either PRDN, 1 mg/kg body weight per day, or a placebo (randomization B), from day 4 to day 21 in blinded manner. R esults Between day 1 and day 21, the number of days on which platelet counts were above 50 × 109/L and above 20 × 109/L was significantly higher among the 56 patients receiving IVIg than among the 60 patients receiving HDMP (P = 0·008 and P = 0·013, respectively), and the percentage of patients who had a platelet count exceeding 50 × 109/L on day 5 was also higher in the IVIg group. During the second treatment period, PRDN was more effective than the placebo on all short-term end points. Patients who received IVIg followed by PRDN had a significantly larger number of days with platelet counts > 50 × 109/L and > 20 × 109/L between day 1 and day 21 (P = 0·008 and P = 0·005, respectively) than patients treated with HDMP followed by PRDN. As regards the long-term response, the percentage of remitters was 40% overall and was not influenced by the treatment allocation in either randomization period (A or B). C onclusion IVIg followed by PRDN appears to be the more effective treatment in adults with severe AITP.

Graves Disease Associated With Autoimmune Thrombocytopenic Purpura

Five patients with active Graves disease had moderate to severe autoimmune thrombocytopenic purpura at the time of diagnosis. Correction of hyperthyroidism and restitution of a euthyroid state by carbimazole therapy resulted in complete normalization of platelet counts.

Thrombocytopenic Purpura as a Manifestation of Acute Hepatitis A

Extrahepatic autoimmune manifestations are rare in patients with acute hepatitis A infection. We describe a 34-year-old man in whom severe autoimmune thrombocytopenic purpura developed as the manifestation of acute hepatitis A infection. Thrombocytopenic purpura is rarely described in association with hepatitis A, but to our knowledge has never been reported as a manifestation of acute hepatitis A.

Autoimmune thrombocytopenic purpura in homosexual men.

Since November 1980 we have diagnosed 11 cases of severe autoimmune thrombocytopenic purpura in homosexual men; their mean platelet count (+/- SE) was 16 000 +/- 3000/mm3. All patients have been sexually active with multiple partners and exposed to numerous viruses and drugs. During this period, we also have diagnosed 20 cases of classic autoimmune thrombocytopenic purpura in heterosexual persons, with a normal women to men ratio of 3:1. Eight of nine homosexual patients had elevated platelet IgG compared with normal values in eight of 10 homosexual control subjects having normal hemograms (p less than 0.01). All responded moderately or completely to steroids. The three patients who had splenectomy had excellent responses. Four of five patients had a decreased helper/suppressor T cell ratio compared to healthy controls (p less than 0.001). Circulating immune complexes and total gamma globulin levels were elevated and lymphocytes relatively decreased in homosexual patients compared with homosexual controls (p less than 0.05). Thus, some sexually-active homosexual men seem to have an increased incidence of an immune regulation disorder directed against platelets.