Kuenstner W, Zhao P, Lee W, Garcia C, Burman KD, et al.
AACE clinical case reports •
Cribriform-morular thyroid carcinoma (CMTC) was considered a variant of papillary thyroid carcinoma (PTC) but is a separate entity in the 2022 World Health Organization classification. CMTC has an association with familial adenomatous polyposis (FAP). Our objective is to report a case of CMTC who was subsequently diagnosed with FAP, to highlight these associated entities and implications for management. A 15-year-old female with a history of iron-deficiency anemia and alpha-gal syndrome presented with several years of goiter and dysphagia. She also noted unintentional weight loss, abdominal pain, melena and hematochezia, and symptomatic anemia. Physical examination was significant for multiple thyroid nodules. Laboratory results revealed normal thyroid function and iron deficiency. Multiple nodules were visualized on thyroid ultrasound, and fine needle aspiration biopsy was consistent with PTC. Total thyroidectomy was performed with a revised diagnosis of multifocal CMTC, with administration of adjuvant radioactive iodine due to persistent disease. Genetic testing confirmed FAP and she was referred for upper endoscopy, colonoscopy, and an evaluation for colectomy. There are no best practice guidelines for management of CMTC. Management of CMTC is guided by FAP status; sporadic cases can be managed with hemithyroidectomy, while FAP-associated cases are better managed with total thyroidectomy. Recurrence is usually managed with surgical resection. The decision to treat with adjuvant radioactive iodine is often extrapolated from management of classic PTC. Thyroid carcinoma in the setting of extensive family history of colorectal carcinoma should arouse suspicion for CMTC. Patients with CMTC should receive a referral for colonoscopy and genetic testing for FAP.
Alpha-gal syndrome (AGS) is a potentially life-threatening acquired meat allergy associated with tick bites. The allergen Galactose-α-1,3-Galactose is antigenically similar to the B blood group antigen. B blood group status offers some protection against development of the allergy. Although not preferred practice, transfusion of plasma and platelets from group B donors is believed to be safe for group O recipients. Anaphylactic transfusion reactions were reported for three patients in two Washington DC hospitals from Nov 2022 to February 2023. A chart review was performed for all three patients. Patients or family members were interviewed, and IgE levels to alpha-gal were measured in two of the three patients. One reaction was acutely fatal. All reactions were to blood group B Plasma or Platelets in blood group O recipients. One patient had two prior anaphylactic reactions to group O RBCs and group B Plasma in a previous admission. All patients came from southern Maryland where AGS is an emerging problem. Two patients had histories of tick bites, previously unexplained gastrointestinal complaints, and abnormal elevated levels of IgE to alpha gal. Two patients had cat allergies. AGS is an emerging problem which may have implications for blood transfusion practice. Avoidance of blood group B antigen containing components may be prudent in non-blood group B patients with established AGS. Investigation for AGS should be considered in the evaluation of anaphylactic transfusion reactions.
Alpha-gal (AG) allergy is an IgE-mediated allergic reaction to galactose-alpha-1,3-galactose found in mammalian meat. Heparin, being derived from porcine intestinal tissue, may have a degree of cross-reactivity with AG antigen and thus place patients at risk for allergic and even anaphylactic reactions. This is especially important in patients with myocardial infarction (MI) and mechanical circulatory support, such as a left ventricular assist device (LVAD), since anticoagulation is immediately required. Therefore, individualized assessment and preoperative planning is needed regarding the use of heparin vs. nonheparinoid products in such a population.
