Alpha 1-antitrypsin (AAT) belongs to the family of serpins (serine protease inhibitors). Loop sheet polymerization is the pathology behind serpinopathies which encompasses AAT, anti-thrombin III and neuroserpin deficiency. To the best of our knowledge, we report the first case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis without any concomitant use of drugs.

Background Frontal QRS-T angle (FQRST) has previously been correlated with mortality in patients with stable coronary artery disease, but its role as survival predictor after ST-elevation myocardial infarction (STEMI) remains unknown. Methods We evaluated 267 consecutive patients with STEMI undergoing reperfusion or coronary artery bypass grafting. Data assessed included demographics, clinical presentation, electrocardiograms, medical therapy, and one-year mortality. Results Of 267 patients, 187 (70%) were males and most (49.4%) patients were Caucasian. All-cause mortality was significantly higher among patients with the highest (101–180°) FQRST [28% vs. 15%, p = 0.02]. Patients with FQRST 1–50° had higher survival (85.6%) compared with FQRST = 51–100° (72.3%) and FQRST = 101–180° (67.9%), [log rank, p = 0.01]. Adjusting for significant variables identified during univariate analysis, FQRST (OR = 2.04 [95% CI: 1.31–13.50]) remained an independent predictor of one-year mortality. FQRST-based risk score (1–50° = 0 points, 51–100° = 2 points, 101–180° = 5 points) had excellent discriminatory ability for one-year mortality when combined with Mayo Clinic Risk Score (C statistic = 0.875 [95%CI: 0.813–0.937]. A high (>4 points) FQRST risk score was associated with greater mortality (32% vs. 19%, p = 0.02) and longer length of stay (6 vs. 2 days, p < 0.001). Conclusion FQRST represents a novel independent predictor of one-year mortality in patients with STEMI undergoing reperfusion. A high FQRST-based risk score was associated with greater mortality and longer length of stay and, after combining with Mayo Clinic Risk Score, improved discriminatory ability for one-year mortality.

A 60-year-old male patient presented to the emergency department with complaints of easy bruising and worsening epistaxis after receiving severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) Moderna mRNA vaccination. He had no personal or family history of hematological conditions. He had bruises in various stages involving the upper and lower extremities. Laboratory data revealed white blood cell count of 1.2 ×10 3 /mm 3 , hemoglobin of 8.0 g/dL, platelet count of 1 ×10 3 /mm 3 , immature platelet fraction of 0.7%, absolute neutrophil count of 0 ×10 3 /µL, lymphocytes of 1.1 ×10 3 /µL, neutrophils of 3% and lymphocytes of 93%. He had normal liver and renal function tests. Bone marrow biopsy confirmed very severe aplastic anemia with severely hypocellular bone marrow. His platelets continued to downtrend despite platelet transfusions and steroids. He was treated with immunosuppressive therapy with cyclosporine, anti-thymocyte globulin, eltrombopag and prednisone. The patient was discharged but was readmitted to the hospital secondary to recurrent neutropenic fever and pneumonia. He had high-grade vancomycin-resistant enterococcal infection and Clostridium difficile infection leading to septic shock and succumbing to cardiac arrest. This case demonstrates the possibility of very severe aplastic anemia following SARS-CoV-2 mRNA vaccination and clinicians need to be aware of this rare but serious side effect.

with tenderness and positive Homan's sign. Deep venous thrombosis (DVT) of left lower extremity with pulmonary embolism (PE) was suspected and lower extremity venous doppler revealed acute extensive DVT in the left femoral, popliteal, gastrocnemius and calf veins. A contrast computed tomography chest scan using PE protocol was suggestive of bilateral pulmonary emboli. Anticoagulation with heparin and coumadin was started simultaneously. The fevers were initially thought to be secondary to thromboembolism but did not abate although patient was on heparin infusion and further evaluation showed atypical lymphocytosis and viral serology for Epstein-Barr virus, Cytomegalovirus and Herpes virus was performed. This revealed evidence of acute Cytomegalovirus (CMV) infection with elevated immunoglobulin M (IGM) of >5.0 and positive polymerase chain reaction (PCR) copies at 5,279 copies/mL. Treatment with ganciclovir for one week resolved his fevers and CMV PCR later became undetectable. Lower extremity pain and swelling improved and the patient was discharged on coumadin.He was followed as an outpatient but he did not experience total resolution of lower extremity symptoms. Venous doppler of left lower extremity showed persistent venous thrombi and he received two sessions of intravascular catheter directed thrombolysis with tissue plasminogen activator, mechanical thrombectomy followed by low molecular heparin therapy (with enoxaparin) and coumadin was discontinued. (Figure 1) shows the venogram of left lower extremity before catheter directed thrombolysis and (Figure 2) shows venogram after catheter directed thrombolysis with improved venous patency.On enoxaparin therapy, he experienced complete resolution of symptoms and was able to restart coumadin. Thrombophilia work up before initiation of anticoagulants showed presence of for lupus anticoagulant, but the repeat testing did not show this. Currently patient is maintained on aspirin and lower extremity compression stockings with no further recurrences of DVT.

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