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Fulminant Rocky Mountain spotted fever. Its pathologic characteristics associated with glucose-6-phosphate
Abstract:
Three patients with documented fulminant Rocky Mountain spotted fever (RMSF) (death on or before day 5 of illness) had severe multisystemic injury as shown by clinical signs and laboratory data, but on microscopic examination showed minimal evidence of the typical mononuclear leukocytic response to rickettsial vascular infection and injury. Thrombosis was more extensive than in classic RMSF, with fibrin thrombi located in foci of rickettsial infection. These patients had a rash either preterminally or not at all, particularly severe Rickettsia-associated pulmonary lesions, and other shock-related lesions, eg, centrilobular hepatic necrosis. All three patients were male blacks with glucose-6-phosphate dehydrogenase deficiency, a condition recently associated with severity of RMSF. Diagnosis of fulminant RMSF requires awareness of its pathologic and epidemiologic aspects, and use of rickettsial isolation or specific immunofluorescence.
Insights
Fulminant Rocky Mountain spotted fever (RMSF) in male Black patients with G6PD deficiency presents with severe injury but minimal leukocytic response. Extensive thrombosis and atypical rash are key pathological findings.
Area of Science:
- Infectious Diseases
- Pathology
- Hematology
Background:
- Rocky Mountain spotted fever (RMSF) is a tick-borne illness caused by Rickettsia rickettsii.
- Fulminant RMSF is a rapidly progressing form with high mortality.
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency has been linked to RMSF severity.
Observation:
- Microscopic examination of three fulminant RMSF cases revealed minimal mononuclear leukocytic response.
- Extensive fibrin thrombi were observed in foci of rickettsial infection, exceeding typical RMSF.
- Patients presented with atypical rash (preterminal or absent), severe pulmonary lesions, and shock-related hepatic necrosis.
Findings:
- Fulminant RMSF in male Black patients with G6PD deficiency exhibits distinct pathological features.
- Atypical inflammatory response and prominent thrombosis characterize severe RMSF.
- The absence of a typical rash and the presence of extensive vascular damage are critical indicators.
Implications:
- Diagnosis of fulminant RMSF necessitates considering specific pathologic and epidemiologic factors.
- Awareness of G6PD deficiency's role in severe RMSF is crucial for clinical management.
- Rickettsial isolation or immunofluorescence are vital for confirming diagnosis in severe cases.