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[Cases of hemophagocytic syndrome in Tenri Hospital]
1Department of Clinical Pathology, Tenri Hospital.
Insights
This study on hemophagocytic syndrome (HPS) found that while low white blood cells (WBC) and elevated C-reactive protein (CRP) are common, they don't reliably predict severity. Diagnosis requires careful consideration of all clinical and lab data.
Area of Science:
- Hematology
- Immunology
- Pediatrics
Context:
- Hemophagocytic syndrome (HPS) is a rare but life-threatening condition.
- Diagnosis can be challenging, relying on a combination of clinical and laboratory findings.
Purpose:
- To review clinical and laboratory findings in patients diagnosed with hemophagocytic syndrome.
- To identify factors associated with HPS and evaluate the utility of common laboratory markers.
Summary:
- This study analyzed 11 HPS cases, noting viral infections as the most common etiology.
- While decreased white blood cells (WBC) and increased C-reactive protein (CRP) were observed, their levels varied widely and did not correlate with disease severity or other indicators like hepatosplenomegaly.
- Poor prognostic indicators included hepatosplenomegaly with lymphadenopathy, disseminated intravascular coagulopathy, elevated alpha 1-globulin, and hyperbilirubinemia.
Impact:
- Highlights the variability of WBC and CRP in HPS, suggesting these markers alone are insufficient for prognosis.
- Emphasizes the importance of bone marrow examination and considering HPS even without overt hemophagocytosis.
- Informs clinical practice regarding the diagnostic challenges and prognostic factors in HPS.
Unlabelled:
The author reviewed clinical and laboratory findings included white blood cells(WBC), C-reactive protein(CRP) in cases of hemophagocytic syndrome(HPS).
Materials:
Eleven cases of HPS treated in Tenri hospital between 1985 and 1999 were studied. HPS was diagnosed by clinical and laboratory findings. There were 6 children(9 months-5 years-old, male 2: female 4) and 5 adults(31-74 years-old, all: female).
Results And Discussion:
Etiology of HPS was viral infection in 8 cases and autoimmune diseases, drug allergy, malignant lymphoma in the other 3 cases. Clinical and laboratory findings that indicated poor prognosis were hepatosplenomegaly complicated with lymphadenopathy, disseminated intravascular coagulopathy, increase of alpha 1-globulin and hyperbilirubinemia. The number of WBC ranged between 500 and 4300/microliter. WBC was decreased in 9 cases and there were cases demonstrating relative lymphopenia or neutropenia. However, CRP increased in all cases (0.2-30.7 mg/dl), although in 8 cases, the CRP values were less than 4 mg/dl. There was no relation between the number of WBC and CRP values and neither of these items showed any relation to hepatosplenomegaly or values of lactate dehydrogenase and ferritin. We should prepare histological sections along with smears from bone marrow examination to increase the sensitivity of diagnosing HPS. We should also note that even if there is no increase in hemophagocytes, we can not deny HPS.
Conclusion:
Cytopenia and increases of CRP, alpha 1-globulin, lactate dehydrogenase and ferritin were common findings in HPS, but there was no relation among these findings and there were large differences in these findings in each case.
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