Document Type
Conference Proceeding
Publication Date
5-3-2024
Abstract
Patients with Waldenstrom's macroglobulinemia/lymphoplasmacytic lymphoma are at increased risk of sinopulmonary infections, pyelonephritis, and septicemia. The underlying etiology is assumed to be immune dysregulation. Patient's with Waldenstrom's macroglobulinemia have been shown to have somatic mutations in the "V". "D" and "J/H' segments of immunoglobulin gene. These mutations can affect affinity maturation and formation of memory B cells. Subsequently patient's with Waldenstrom's macroglobulinemia also have a suboptimal response to vaccination against Staphylococcus aureus, Streptococcus pneumoniae and Varicella, similar to those with multiple myeloma. Severe infections with gram positive organisms causing multifocal osteomyelitis, pleural effusion and sepsis have also been reported with Waldenstrom's macroglobulinemia 64-year-old female with past medical history of recently diagnosed Waldenström's macroglobulinemia/chemical plasmacytic lymphoma presented to the emergency department with complaints of pain and swelling in the neck. Around 3 weeks prior to presentation, patient noted a swelling in the left side of the neck after she accidentally punctured the neck of her skin with her fingernail. She went to the ER but workup was negative for acute infection. Contrast-enhanced CT scan of the neck showed multiple enlarged bilateral cervical lymph nodes. Around 14 days after the the incident, patient started developing painful swellings in the neck. The pain was sharp in nature, 10/10 in intensity localized to the area of swelling. In the ER, patient was tachycardic and afebrile. Examination revelaed a visible 3x3 cm swelling in the neck, with overlying redness, warmth and tenderness to palpation. Lab investigations were significant for lymphocytic leukocytosis with WBC of 31.5 (lymphocyte %;24.8, neutrophil% 6.1). Contrast-enhanced CT scan of the neck 5x5 centimeter ring-enhancing lesion in the subclavicular space at the junction of the proximal 2/3rd and distal 1/3rd of the clavicle. Patient underwent US guided drainage of abscess with drain placement. Abscess fluid cultures were positive for Streptococcus pneumoniae. Patient had history of vaccination with PCV 13 around 18 years ago Patient was treated with IV ceftriaxone followed by oral cephalexin for total of 14 days. She also underwent repeat vaccination with PCV, meningococcal and hemophilus influenzae vaccine after resolution of infection. Of note, patient's previous PET scan showed marked splenomegaly with increased FDG intake While neutropenia has been known as a cause of recurrent infection in B cell neoplasms, the cause of recurrent infection in patients with a normal neutrophil count remains elusive. The theoritical explanation of immune disregulation due to genetic mutations have not been demonstrated in vivo. Moreover, abscess formation in a vaccinated individual highlights the decreased ability of patient's with Waldenstrom's macroglobulinemia to mount an effective antibody response. Splenic involvement in patients with plasma/ B cell disorders further increases chances of infection possibly secondary to alteration in antibody opsonisation. Defective opsonisation is a known complication of multiple myeloma; as a result patients with multiple myeloma are predisposed to sepsis with streptococcal pneumoniae, klebsiells pneumoniae and other encapsulated organisms. It is possible that a similar mechanism predisposes patient's with Waldenstrom's macroglobulinemia to infections with encapsulated organisms. It is important to ensure resolution of localised infection to prevent bacteremia.
Recommended Citation
Amal T, Johnson P, Sharif A. Streptococcus pneumoniae neck abscess in a patient with Waldenstrom's macroglobulinemia. Presented at: American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2024 Resident and Medical Student Day; 2024 May 3; Troy, MI
Comments
American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2024 Resident and Medical Student Day, May 3, 2024, Troy, MI