Postpartum inhibitor to factor VIII: treatment with high-dose immunoglobulin and dexamethasone.

Spontaneous occurrence of an acquired inhibitor to factor VIII (FVIII) is a rare event. About 50% of cases are idiopathic. Among younger people, inhibitors are often found in the postpartum period. Treatment must be administered either to overcome haemorrhagic symptoms or to eradicate the inhibitor. Several approaches have been proposed for inhibitor eradication, based on immunosuppressive drugs such as corticosteroids, cyclophosphamide and azathioprine, with varying results. High-dose immunoglobulin (HDIg) has been recently proposed as first-line therapy. We report on four cases with acquired inhibitor to FVIII occurring 4-8 months after delivery. At diagnosis, inhibitor titre was < 5 Bethesda units mL(-1) (BU mL(-1)) in three cases and > 5 BU mL(-1) in one. Factor VIII coagulant activity (FVIII:C) was < 1 U dL(-1)> in three cases and 12 U dL(-1) in one. We treated the patients with HDIg (400 mg kg-1 day(-1) for 5 consecutive days) and dexamethasone (24 mg day(-1) for 5-7 consecutive days), administered at the same time. In three women, the inhibitor was suppressed in 2-50 weeks. After an off-therapy period ranging from 20 to 104 weeks, the FVIII:C was persistently normal and the inhibitor undetectable. The fourth woman remained unresponsive. In two cases, recombinant activated factor VII administration stopped the bleeding. Thus, intermediate- to high-dose dexamethasone and HDIg given at the same time could be a successful and safe therapeutic approach for a rapid and complete remission from the development of FVIII inhibitors.

Long-term follow-up of autoimmune thrombocytopenic purpura (ATP) patients submitted to splenectomy.

We studied the general outcome in 94 adult patients with autoimmune thrombocytopenic purpura (ATP) submitted to splenectomy. Of 84/94 patients who presented a complete or partial response 30 d after splenectomy, 16 (19%) showed one or more relapses. The clinical situation of the 81 patients still under observation is as follows: 13 unresponsive, 60 completely or partially responsive, without relapses during the follow-up, 8 completely or partially responsive after one or more relapses. No correlation was found between the favourable splenectomy outcome and age at splenectomy, the diagnosis-splenectomy interval and initial response to corticosteroids. The probability of disease-free survival is 83%, projected at 10 yr, while the overall survival is 93%, projected at 10 yr. PAIgG levels of the normal subjects and of responding patients were found to be similar, while in the groups of non-responding/relapsing patients, significantly higher values of PAIgG were detected, as compared to the control group.

Acquired inhibitor against factor IX in a child: successful treatment with high-dose immunoglobulin and dexamethasone.

The occurrence of acquired inhibitor against factor IX:C is infrequent in haemophilia B patients and is very rare in previously healthy subjects, in whom it is often related to underlying diseases. We describe the case of a 2-year-old girl, who was referred to our hospital with haematomas, without previous bleeding history. Prolonged APTT, normal PT and a factor IX:C level below 1% were found. An inhibitor against factor IX:C was detected (5.5 U mL(-1)). Her father and mother showed normal factor IX:C levels. Treatment with high-dose immunoglobulin (400 mg kg(-1) day(-1) for 5 consecutive days by intravenous infusion) and dexamethasone (4 mg three times a day by intravenous injection for 4 consecutive days) normalized factor IX:C levels and overcame the inhibitor. In conclusion, high-dose immunoglobulin and high-dose dexamethasone are a successful and safe immunosuppressive approach for recovery from inhibitor occurrence.

Rapid liver failure related to chronic C hepatitis in an HIV seropositive hemophilic patient with severe immunodepression.

We report the case of a young HIV seropositive patient with severe hemophilia A who presented rapid liver failure related to his chronic C hepatitis. The patient had been receiving factor VIII:C clotting factor concentrates (mean 60,000 U/year) since 1975. In 1984 alanine aminotransferase presented abnormal levels. The CD4 lymphocyte count in 1991 was normal and ultrasonographic scan showed normal liver morphology. In 1991 the patient were found to be seropositive for HCV antibodies as detected by the ELISA method and confirmed by the RIBA method. One year later, a progressive increase in policlonal gamma-globulin and a decrease in the CD4+ lymphocyte count to below 500/muL were detected in concomitance with ultrasonographic evidence of a progressive increase in the longitudinal diameters of the liver and spleen and signs of liver inhomogeneity. A significant inverse correlation was observed between the increase in the longitudinal diameter of the liver and the decline in albumin levels, and between the increase in the longitudinal diameter of the liver and the drop in platelet count. Elevated levels of ammonemia, gamma-glutamyl transpeptidase, alkaline phosphatase and IgA were detected. Moreover, decreased levels of the C4 and C3 complement fractions were documented. At this time (1994), esophagogram and esophagogastroscopy evidenced varicosities in the lower esophageal section (stage F1). The patient died in 1995 March at the age of 29 years of sudden septic shock related to Pseudomonas aeruginosa infection.

Auditory syntactic comprehension in nine aphasia groups (with CT scans) and children: differences in degree but not order of difficulty observed.

Auditory syntactic comprehension was examined in nine groups of aphasics (60 stroke cases) and 3 and 6 year old children. Ten syntactic contrast pairs were studied. Differences in degree but not order of difficulty were observed. The 5 easier pairs were Gender; On/Under; Negative/Affirmative; Object-number; Subject-number; the 5 more difficult pairs were Past/Present; Subject/Object Reversible; Is/Are; Relative Clause; and Future/Present. The "marked" half-pair was significantly more difficult--i.e., Negative vs. Affirmative; Plural vs. Singular Subject; Future or Past vs. Present. Performance on the test was significantly correlated with the BDAE Auditory Comprehension z-score and Token Test. The 3 year old children most closely resembled the Severe Wernicke's (but were better than the Globals), and the 6 year old children were similar to aphasia cases with mild comprehension deficits and frontal or parietal perisylvian lesions which spared most of Wernicke's area. Wernicke's area (posterior two-thirds superior temporal gyrus area) appears to be the area most sensitive to auditory syntactic comprehension. However, the surrounding perisylvian areas including frontal and parietal lobes are also areas sensitive to syntactic comprehension.