Emicizumab Use in Treatment of Acquired Hemophilia A: A Case Report.

BACKGROUND Acquired hemophilia A (AHA) is a rare hemorrhagic disorder that is caused by producing autoantibodies against factor VIII. It is usually characterized by severe, spontaneous bleeding, which can be life-threatening. The current standard treatments for bleeding prophylaxis are highly effective but accompanied with some disadvantages such as frequent intravenous infusions, high cost, and risk of thromboembolic complications. Emicizumab is a bispecific antibody with a therapeutic FVIII-mimetic nature. Emicizumab has shown a reduction in annualized bleeding rate in congenital hemophilia patients with and without inhibitors. The pathophysiological concepts and preclinical data suggest that Emicizumab can be effectively used for treating AHA. CASE REPORT We present the case of an 87-year-old woman admitted for symptomatic anemia and large chest wall and pelvic hematomas confirmed by imaging, without history of trauma. Her coagulation studies showed isolated prolonged activated partial thromboplastin time (aPTT), low factor VIII activity level, and high levels of factor VIII inhibitor. She was successfully treated with activated prothrombin complex concentrate (aPCC), which was transitioned to Emicizumab on discharge. No recurrent bleeding episodes or adverse events related to Emicizumab were reported during the 2-month follow-up period. CONCLUSIONS A subcutaneous weekly or biweekly injection of Emicizumab, a recombinant monoclonal antibody, offers several advantages: less frequent infusions, good hemostatic efficacy, possible outpatient therapy, and even more cost-effective than bypassing agents. More clinical studies should be conducted to compare Emicizumab with the current standards of care.

Relapsing Cutaneous Multiple Myeloma Responding to Immunochemotherapy: A Rare Case Report.

Multiple myeloma (MM) is characterized by the neoplastic proliferation of plasma cells. The diagnosis of this disease is often suspected through a constellation of clinical signs and symptoms of hypercalcemia, renal failure, anemia, and M-spike proteins. It is the second most common hematological malignancy after non-Hodgkin lymphomas. However, cutaneous MM is an extremely rare entity, and it is associated with poor prognosis. It presents as diffuse erythematous rash or violaceous nodules on the skin. Most common sites of involvement are chest, lower extremities and back. It can be triggered by a local extension of the tumor which is the most common way, surgical procedures and hematogenous spread. An 82-year-old African American male was diagnosed with MM since 2008. He underwent autologous peripheral stem cell transplantation (ASCT) twice in 2010 and 2014; and he had a history of multiple chemotherapy regimens in the past. He had violaceus chest nodules, and the biopsy confirmed the diagnosis of cutaneous MM in 2013. The patient was treated with pomalidomide, panobinostat and dexamethasone with a complete response (CR) to treatment. One year later, the patient developed new skin nodules. Repeat biopsy confirmed the diagnosis of MM again. Patient was treated with daratumumab and had CR to treatment without any new M-spike. Cutaneous lesion is an exceedingly rare presentation of MM. It either present as reddish rash or violaceous nodules involving chest, lower extremities and back. It has a poor prognosis and can be rapidly fatal. Our case is unique because our patient responded to the newer chemotherapy, and lesions resolved despite poor prognosis of this condition.

Dabigatran-Induced Acute Interstitial Nephritis: An Important Complication of Newer Oral Anticoagulation Agents.

Acute kidney injury (AKI) due to an acute interstitial nephritis (AIN) is common and can lead to increased morbidity and mortality. Medications such as antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), proton pump inhibitors (PPI) and rifampin are common offending agents. Anticoagulant-associated AIN is more frequently reported with the use of warfarin; however, only few case reports have reported an association with the use of novel oral anticoagulants (NOACs). Herein, we report the case of a 59-year-old male who developed AKI after initiating dabigatran for the treatment of atrial fibrillation. Laboratory data demonstrated elevated blood urea nitrogen (BUN) of 115 mg/dL (baseline = 35 mg/dL) and serum creatinine (Cr) of 5.06 mg/dL (baseline = 1.3 mg/dL). Urinalysis revealed eosinophiluria. Renal biopsy disclosed diffuse tubulointerstitial nephritis and eosinophils and confirmed the diagnosis of AIN. At 1 week, renal function improved (BUN/Cr = 53/2.73 mg/dL) with steroid therapy and discontinuation of dabigatran. With an increasing use of NOACs, it is important to monitor renal function to diagnose AIN in a timely fashion. Early diagnosis and prompt treatment can mitigate serious renal damage induced by dabigatran.