(P-TS-11) An unexplained fatal catastrophic anaphylactic reaction during plasma exchange and subsequent follow-up

Anaphylactic transfusion reaction (ATR) is a rare entity, with an incidence of 1 in 20,000-50,000. Symptoms can include hypotension, mucocutaneous manifestations, dyspnea, and even death. From 2017-2021, ATR comprised 9% of transfusion-associated fatalities in the United States. The mechanisms of ATR may include; passive transfusion of allergen or pre formed-IgE, biologic response modifiers and concomitant exposure to another allergen.

Study

Design/Methods:

We present a 65-year-old male with a history of seafood allergy, chronic obstructive pulmonary disease (COPD), congestive heart failure (CHF) and Waldenstrom's macroglobulinemia/Lymphoplasmacytic Lymphoma (WM/LPL). The patient received multiple plasma exchanges and numerous blood products without reported incidents. He presented to our institution emergency department with shortness of breath and headaches. His admission laboratory tests revealed evidence of hyperviscosity syndrome including IgM 5,5850 mg/dl and serum viscosity 3.0 Centipoise (cP), necessitating an emergent plasma exchange.

The plasma exchange planned to utilize 5% albumin and partial plasma (30%) due to elevated PT/INR. The albumin phase of the exchange was uneventful. After starting the plasma phase and toward the end of the first plasma unit, the patient became restless, short of breath and very anxious. The exchange was paused, however immediately the patient became severely hypoxic and unresponsive. Cardiopulmonary resuscitation was started; but it was unsuccessful.  Transfusion reaction evaluation was initiated per protocol.

Results/Findings:

Routine laboratory workup ruled out hemolytic transfusion reaction. Transfusion-related acute lung injury (TRALI) type II, pulmonary embolism and ATR were the initial impression given the acute onset of respiratory distress. TRALI investigation by our institution blood donor center was negative. Post mortem examination was performed by our institution and was inconclusive. The post-exchange tryptase level was profoundly elevated 287.0 ng/ml (pre-exchange - 7.7 ng/ml) confirming ATR. IgA and haptoglobin deficiencies were ruled out. Additionally, the plasma donor was interviewed, and he denied any history of allergy or consuming seafood prior to the donation, exclusion the potential passive transfusion of pre formed IgE or allergen.

Conclusions:

We present a challenging case of catastrophic anaphylaxis that resulted in a transfusion related fatality. Deaths from ATR are very rare, the pathogenesis is not fully understood, although multiple mechanisms have been postulated, none elucidates our case. A tryptase value above 45 ng/ml had been used to discriminate anaphylactic from nonanaphylactic deaths.  Prompt labs and a thorough post-mortem investigation aided us to ascertain the cause of death in this unfortunate case.