Related Experiment Videos
Inapparent polycythemia vera: an unrecognized diagnosis
T Lamy1, A Devillers, M Bernard
1Department of Hematology, Hôpital Pontchaillou, Rennes, France.
The American Journal of Medicine
|January 1, 1997
Summary
Inapparent polycythemia vera (IPV) is often missed, with normal hemoglobin and hematocrit masking increased red cell mass. Early diagnosis requires considering specific symptoms like splenomegaly or thrombosis.
Area of Science:
- Hematology
- Oncology
Background:
- Polycythemia vera (PV) diagnosis relies on established criteria.
- Plasma volume (PV) expansion can mask red cell mass (RCM) increases, leading to inapparent polycythemia vera (IPV).
Purpose of the Study:
- To investigate the characteristics and diagnostic challenges of inapparent polycythemia vera.
- To evaluate the utility of current diagnostic criteria in identifying all PV cases.
Main Methods:
- Studied 103 patients diagnosed with polycythemia vera.
- Classified patients based on hemoglobin (Hb) and hematocrit (Ht) levels.
- Confirmed PV diagnosis using Polycythemia Vera Study Group (PVSG) criteria, spontaneous erythroid colonies, and low serum erythropoietin.
Main Results:
- 85 patients had overt PV (Group A) with elevated Hb/Ht.
- 18 patients (17%) had inapparent polycythemia vera (IPV) (Group B) with normal Hb/Ht.
- IPV diagnosis was prompted by splenomegaly, portal vein thrombosis, or elevated platelets/leukocytes.
- Group B showed significantly higher PV increase (+36.3%) compared to Group A (+9.5%).
Conclusions:
- Increased Hb or Ht should prompt RCM determination.
- RCM testing is crucial in cases of portal vein thrombosis, isolated hyperleucocytosis, thrombocytosis, or splenomegaly.
- The frequency of IPV is likely underestimated, suggesting PV diagnosis may be underreported.