Azmanira Binti Aziz has completed her Medical degree from Universiti Putra Malaysia, Malaysia. Currently, she is pursuing her Master’s MMed in Transfusion Medicine at Universiti Sains Malaysia. She has been working in National Blood Centre, Kuala Lumpur since 2010 to till date.
Inhibitor formation is one of the primary complications in the treatment of hemophilia A. Immune Tolerance Induction (ITI) has been used to eliminate inhibitor. This retrospective study described pediatrics patients who had received ITI therapy at the National Blood Centre Kuala Lumpur (NBCKL). This study is undertaken by reviewing the files of patients diagnosed as hemophilia A with inhibitors underwent ITI therapy at the NBCKL from 2002 to June 2016. ITI therapy was given initially 50-100 IU/kg/d and the dose was reduced based on the inhibitor titer and FVII recovery level. The successful outcome was defined as negative titer inhibitors and FVIII level were normalized. Twelve (66.6%) patients out of 18 from this study successfully eliminate inhibitors. This study also showed statistically significant association with the historical peak inhibitor before start ITI (p=0.015), peak of inhibitor during ITI (p=0.018), inhibitor titer before ITI (p=0.036), inhibitor at the start of ITI (p=0.011) and the duration between inhibitor detection and the start of ITI (p=0.046) with the final outcome of ITI therapy. This study has demonstrated that ITI therapy can be an effective treatment as inhibitor eliminator. The successful ITI factors includes the history of low inhibitor titer peak prior to therapy and peak during ITI (<200 BU/ml), low inhibitor titer before and at start of ITI (<10 BU/ml). The shorter duration of starting ITI therapy after inhibitor detection could influence the ITI outcome with higher success rate.
Kavita Agrawal completed her medical school from Pramukhswami Medical College, India in 2014. Currently, she is a second year resident in Internal Medicine at Overlook Medical Center, New Jersey. She has recently published an article ‘Association of Autoimmune hemolytic anemia and gastric adenocarcinoma’. She hopes to purse a fellowship in Hematology and Oncology after her residency.
We present a case of a 48-year-old male who presented with worsening pleuritic chest pain for two days. He also complained of fever, malaise, headache and severe neck pain. On admission, electrocardiogram (ECG) showed ST segment elevation in leads I, II, aVL and V5 with PR elevation and ST depression in aVR. Troponin-I was 14.8 ng/ml. Based on the ECG changes, elevated troponin and family history of early coronary artery disease, patient was emergently taken to cardiac catheterization lab. Angiography showed non-obstructive coronaries, mild hypokinesis of mid inferior and anterolateral wall with ejection fraction (EF) of 40-45%. Based on the above presentation and angiographic findings, the diagnosis of acute myopericarditis was made. He was started on colchicine and ibuprofen. Other workup to determine the etiology of myopericarditis was negative (shown in detail in the presentation). Given the history of fever, headache and worsening neck pain, we became suspicious of meningitis. Lumbar puncture was performed which was negative. On the day of admission, he was found to have blasts on complete blood count and peripheral smear. Bone marrow biopsy and flow cytometry confirmed the diagnosis of acute myeloid leukemia (AML). He received induction and salvage therapy. Repeat bone marrow confirmed complete remission and normal cytogenetics. Although pericardial or myocardial biopsies are unavailable for our patient, in the absence of other causes, it does appear that his acute myopericarditis was secondary to AML. Our case highlights pericarditis as an initial manifestation of AML which is a rare phenomenon.