Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in the setting of systemic lupus erythematosus with collapsing lupus podocytopathy superimposed on mesangial proliferative lupus nephritis class II. Genotyping for APOL1 risk alleles revealed the presence of double (G1/G2) risk alleles. Our patient achieved a complete renal recovery and resolution of HLH within 1 month of treatment with steroids and mycophenolate mofetil, highlighting the importance of prompt, aggressive therapy.

Coronary artery ectasia (CAE) can present as an acute coronary syndrome (ACS) with a high clot burden in ectatic coronary arteries. Thrombectomy with intracoronary thrombolysis often does not ensure immediate blood flow. Also, there have not been clear guidelines regarding long-term management in such cases.A 40-year-old male presented with anginal chest discomfort and a working diagnosis of non-ST elevation myocardial infarction (NSTEMI) was made. The initial angiography showed thrombotic occlusion of several large and ectatic coronary arteries with visibly swirling blood flow. The culprit lesions were treated with balloon angioplasty and multiple rounds of thrombectomy yielding red thrombi. Interestingly, the post-intervention antegrade flow decreased in both vessels (Thrombolysis in Myocardial Infarction (TIMI) score: 0), possibly because of the distal migration of the clots. Peri-procedure, the patient received two boluses of eptifibatide, 180 mcg/kg each, followed by a continuous infusion of 2 mcg/kg/minute for 18 hours. Afterward, the patient was started on ticagrelor and continued on daily aspirin, high-intensity statin, beta blocker, and Coumadin® with heparin bridge. During the one year follow-up period, the Coumadin was switched to rivaroxaban, ticagrelor was stopped after six months, and the patient was continued on guideline-directed medical therapy (GDMT) for coronary artery disease (CAD) with favorable outcomes.The presented case gives us an insight into not only the intra-procedural but also the post-procedural management of ACS in the setting of CAE, and that is thrombectomy alone followed by longer duration oral anticoagulation in addition to GDMT for CAD. However, it will be interesting to see future studies aimed toward defining the duration as well as the choice of anticoagulation, i.e., dual antiplatelet therapy (DAPT) alone or in combination with warfarin/novel oral anticoagulants (NOACs).

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