Like in our case, a common scenario includes ineffective immunosuppressive (steroids) and intravenous immunoglobulin-G treatment, unnecessary repeated platelet and cryoprecipitate transfusions, multiple painful bone marrow examinations, and eventually ineffective, unnecessary, and potentially harmful splenectomy for steroid-resistant ITP

Like in our case, a common scenario includes ineffective immunosuppressive (steroids) and intravenous immunoglobulin-G treatment, unnecessary repeated platelet and cryoprecipitate transfusions, multiple painful bone marrow examinations, and eventually ineffective, unnecessary, and potentially harmful splenectomy for steroid-resistant ITP.26,27 Program transfusions of platelet concentrates are used in patients with MHA, to transiently increase platelet count. in leukocytes can differentiate MHA from idiopathic (immune) thrombocytopenic purpura (ITP).1C5 Top of Form MYH9RDs are considered very rare. The Italian Registry for MYH9RD indicates that this prevalence of the disorder in Italy is at least 3:1,000,000.6 Because mild forms are discovered incidentally and severe forms are often misdiagnosed or underreported as other disorders, the actual prevalence is expected to be higher. MYH9RD has been diagnosed worldwide, and there is no evidence of variance in prevalence across ethnic populations.7 In general, the worldwide incidence of MHA is unknown. Thrombocytopenia occurs in approximately 50% of the patients with MHA, and the clinical manifestations vary from moderate bleeding not requiring specific treatment up to severe bleeding episodes following trauma or surgery that require blood products.1C3,8 However, despite severe thrombocytopenia in most patients, platelet function is frequently normal. Therefore, they are asymptomatic, discovered incidentally.1C3,8 Thrombocytopenia is common during pregnancy; however, MHA is very rare, with 40 cases reported in the literature.9 Diagnosis has been established prior to pregnancy in some women, but in the majority, the problem is first identified incidentally during pregnancy as thrombocytopenia on routine booking blood tests. Most of such cases, without careful inspection of blood smears and a thorough family and bleeding history, are in the beginning misdiagnosed as refractory-to-treatment ITP.9 Consequently, the most serious impacts of this disease are iatrogenic managements due to misdiagnosis.3 The diagnosis of MHA may pose a challenge for clinicians managing pregnant women with thrombocytopenia. Herein, we statement a case of MHA in a woman who experienced a successful labor and delivery under epidural anesthesia. The patient provided written informed consent for her data to be included in this case statement. Case description The history of the patient starts 10 years ago on April 2006, when she offered as primigravida at 36 weeks gestation, booked for antenatal investigations. Patient was not known to have any medical illness or history BS-181 HCl of previous surgical procedures. Her platelet count was found to be 22109/L, and she was admitted as a case of possible ITP. Coagulation profile was within normal limits, and there was no history of bleeding tendency. Family history positive for bleeding disorders was not reported. She was started on steroid therapy (in the beginning with prednisolone and later with dexamethasone); however, the platelet count decreased to 19109/L. After transfusion of 6 models Rabbit Polyclonal to VIPR1 of platelets, her platelet count was 20109/L. Then, she received four cycles of rituximab (monoclonal antibody) and three doses of immunoglobulin, without response. Bone marrow biopsy revealed thalassemia trait and iron deficiency. Megakaryocytes and erythroblasts exhibited significant dysplasia. Differential diagnosis was between ITP and myelodysplastic syndrome. She experienced an uncomplicated cesarean section BS-181 HCl under general anesthesia, due to failure to labor progress. She received 18 models of platelets and 6 models of cryoprecipitate since the cesarean section. No bleeding tendency was observed perioperatively. Postoperatively, her platelet BS-181 HCl count was 65109/L, with normal coagulation profile. She was discharged without any complication. After 3 months, a second bone marrow biopsy did not reveal additional pathology and confirmed the initial diagnosis (ITP). Her platelet count was 18109/L, so the patient was scheduled for splenectomy due to refractory ITP. In August 2006, she was transfused with 8 models of platelets and underwent an uncomplicated laparoscopic splenectomy under general anesthesia, with minimal blood loss. The spleen pathology statement was consistent with the clinical diagnosis of ITP..