Paroxysmal Cold Hemoglobinuria in an Adult with Respiratory Syncytial Virus

Abstract

Paroxysmal cold hemoglobinuria (PCH) is a rare form of cold autoimmune hemolytic anemia first discovered in the early 20th century in adults with tertiary syphilis. Today, it is more commonly seen in children as a life-threatening anemia during a viral upper respiratory tract infection (URI). Although respiratory syncytial virus (RSV) has previously been reported to cause PCH in a child, herein we present the first documented case in an adult. The Donath–Landsteiner (DL) test, the diagnostic test for PCH, was positive on two separate occasions. The patient was treated successfully with warming and avoidance of cold temperatures. To facilitate identification of this rare entity by clinicians, we include a discussion about the pathophysiology, diagnosis, and treatment of PCH.

Left Out in the Cold

“…Implicated triggers include a number of viruses. 9 Clinical presentation includes findings of intravascular hemolysis similar to those in our patient. The pathogenic IgG autoantibody is polyclonal and is likely formed because of immune stimulation, which is consistent with the predominance of nonmalignant triggers of this disease process.…”

Autoimmune hemolytic anemia: current knowledge and perspectives

“…PCH is a rare disease which mostly affects children [23]. It is extremely rare in adults and is often associated with infections in this age group [24]. The risk of AIHA increases with age, in wAIHA the risk is 5 times higher in the 7th decade of life compared to the fourth decade [21].…”

A Rare Case of Parvovirus B19 Infection Induced Paroxysmal Cold Hemoglobinuria in an Adult Female

“…The mechanism of how an infectious agent induces PCH is poorly understood. One proposed theory suggests that viruses alter the glycoproteins on the erythrocyte membrane which further stimulates autoantibody formation [ 4 ]. Another theory points that molecular mimicry between self-antigens and foreign antigens leads to the production of cross-reactive antibodies [ 4 ].…”

“…Because of the biphasic nature, the direct antiglobulin test (DAT) is negative for IgG and positive for anti-C3 [ 4 ]. Cold agglutinin disease is one of the main differential diagnoses.…”

“…PCH can occur in both acute and chronic forms. The important risk factors for acute PCH include viral infections (mumps, measles, chickenpox, Epstein-Barr virus, cytomegalovirus, influenza, parvovirus B19, coxsackievirus A9, and adenovirus) and vaccination (measles) [ 4 , 5 ]. The occurrence of parvovirus B19 infection predisposing to acute PCH in adults is rare.…”