Rapidly fatal Klebsiella pneumoniae sepsis in a patient with

pyruvate kinase deficiency and asplenia

Image ID: 61197

Authors: Mohammad Faizan Zahid; Ashish Pal Singh Bains

Category: Infectious Disease > Bacteria > Klebsiella and Pseudomonas

Description: A 24-year-old man presented to the emergency room with respiratory

distress and altered mental status. He had a past medical history of pyruvate kinase
deficiency with status postsplenectomy at 6 years of age. On examination, he was
hypotensive and febrile; he was intubated on admission. Workup showed
macrocytic anemia, leukocytosis, and slight neutrophilia, with elevated lactate,
prothrombin time, and activated partial thromboplastin time, mixed respiratory and
metabolic acidosis, and hypoxemia. Chest radiograph demonstrated dense bilateral
pulmonary infiltrates. Peripheral blood smear showed leukoerythroblastosis with
left-shifted granulocytes exhibiting toxic morphology and numerous extracellular
and intracellular bacilli (Wright stain; original magnification ×100). Additional
findings compatible with asplenic status were also noted, including numerous
nucleated red blood cells, Pappenheimer bodies, and Howell-Jolly bodies (arrows).
Despite broad-spectrum antibiotics, supportive care, and multiple vasopressors, he
remained in intractable septic shock and unfortunately died. Blood cultures grew
Klebsiella pneumoniae that was pan-sensitive.Asplenic patients lack splenic
macrophages necessary for eliminating opsonized encapsulated organisms.
Prophylactic vaccination, particularly with pneumococcal vaccine (which the patient
had received), is important to prevent some life-threatening infections in asplenic
patients. However, vaccination is currently unavailable for K pneumoniae and it
remains one of the common causes of these rare, overwhelming, and rapidly life-
threatening infections in patients who are splenectomized for congenital hemolytic
anemias.

AMERICAN SOCIETY OF HEMATOLOGY

2021 L Street NW, Suite 900, Washington, DC 20036 | Phone 202-776-0544 | Fax 202-776-0545

Copyright © 2020 American Society of Hematology

Copyright © 2020 American Society of Hematology. Copyright restrictions may apply.

AMERICAN SOCIETY OF HEMATOLOGY

2021 L Street NW, Suite 900, Washington, DC 20036 | Phone 202-776-0544 | Fax 202-776-0545

Copyright © 2020 American Society of Hematology