Abstract Introduction Purpura fulminans (PF) is a rapidly progressive thrombotic vasculopathy characterized by purpuric skin necrosis, shock, and disseminated intravascular coagulation (DIC). It typically follows sepsis from encapsulated bacteria and is highly relevant to critical care because early recognition and pathogen-directed therapy must proceed in parallel with aggressive organ support. Case Presentation A 61-year-old man with small lymphocytic/chronic lymphocytic leukemia (SLL/CLL) on ibrutinib presented in septic shock with hypoxic respiratory failure, acute kidney injury, and elevated liver enzymes. He was emergently intubated, started on broad-spectrum antibiotics and vasopressors, and admitted to the ICU. Examination revealed rapidly spreading, violaceous, acral-predominant retiform purpura of the face and all extremities without ocular or mucosal detachment. Initial laboratories showed platelets 57 × 10³/µL, international normalized ratio (INR) 2.6, fibrinogen 40 mg/dL, lactate 16.2 mmol/L, arterial pH 7.01, and elevated D-dimer (20 µg/mL). Blood cultures grew Streptococcus pneumoniae (serotype 3); antibiotics were narrowed accordingly. Despite hemodynamic resuscitation, he developed oligo-anuric renal failure requiring continuous renal-replacement therapy. By hospital day 6, purpuric changes involved approximately 50% total body-surface area with hemorrhagic bullae and superficial desquamation, prompting transfer to a regional burn ICU. Bedside biopsy demonstrated intact epidermis, arguing against Stevens-Johnson syndrome/toxic epidermal necrolysis; formal histopathology showed widespread intravascular thrombi with epidermal and adnexal necrosis, consistent with PF in the setting of DIC. Peripheral blood smear (PBS) revealed approximately 15 schistocytes per high-power field and Howell-Jolly bodies, indicating functional asplenia. Findings supported DIC rather than thrombotic thrombocytopenic purpura; plasmapheresis was not pursued. Despite maximal critical care, he progressed to refractory shock and multiorgan failure, and the family elected comfort measures. Discussion PF is an extreme ICU phenotype of sepsis-induced microvascular injury and consumptive coagulopathy. Functional asplenia from presumed leukemic infiltration disrupting splenic architecture and immune function caused impaired clearance of encapsulated organisms and predisposed to overwhelming pneumococcal sepsis. For intensivists, recognition of hyposplenism at admission is actionable: a routine PBS review for Howell-Jolly bodies can trigger pathogen-directed therapy, early infectious diseases and dermatology involvement, and consideration of burn center support. Additionally, patients with CLL or other causes of functional asplenia warrant systematic prevention strategies, including ensuring up-to-date vaccines against encapsulated organisms and individualized plans for rapid evaluation and empiric antibiotics at first signs of infection. Conclusions Functional asplenia should be recognized as a critical risk factor for severe pneumococcal sepsis and PF. Early detection and preventive measures may improve outcomes in immunocompromised patients presenting with septic shock. This abstract is funded by: None
Macy et al. (Fri,) studied this question.