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A blood test developed to diagnose a rare genetic blood cell disorder, atypical hemolytic uremic syndrome (aHUS), may also aid in the diagnosis of HELLP syndrome, a life-threatening high blood pressure condition that affects 1% of all pregnant women.
The study, based on blood samples from a small number of women, suggests that aHUS has similar underlying biochemistry to HELLP, which affects hemolysis, elevates liver enzymes, and causes a low platelet count. Both conditions have over activation of the alternative pathway of complement.
At present, no diagnostic blood or biomarker test exists to diagnose HELLP, which is thought to be a severe form of preeclampsia. The condition is diagnosed only by its symptoms.
Senior study author Robert Brodsky, MD, of Johns Hopkins, and his team developed the modified Ham test to diagnose aHUS, a genetic disorder in which abnormal blood clots form in small blood vessels in the kidneys. They published that work last year in Blood.
The two conditions share a number of traits, such as hemolysis, elevated liver enzymes, a low platelet count, kidney dysfunction, high blood pressure, and seizures. This led the investigators to believe that the modified Ham test could also help identify women with HELLP syndrome.
"The clinical implications from an obstetric point of view are potentially huge," said lead study author Arthur Vaught, MD, also of Johns Hopkins. "If this works, we can reduce pre-term deliveries, stays in the neonatal intensive care unit, and other complications for mothers and their babies."
The team analyzed serum samples from 14 women with classic or atypical HELLP syndrome, 7 with severe preeclampsia, 11 women with normal pregnancies, and 8 healthy nonpregnant women. All pregnant women were at least 23 weeks’ gestation, the point at which HELLP symptoms start to arise.
The team evaluated patient sera using terminal product of complement activation (C5b-9). They observed that women with classic or atypical HELLP had increased complement activation compared to nonpregnant controls.
Women with classic HELLP had an average cell killing of 34.3% compared with 26% in women with atypical HELLP, 5% in women with normal pregnancies, and3.3% in women who were not pregnant.
The investigators then added eculizumab to HELLP sera to see whether the agent could inhibit complement activation. They found that mixing HELLP serum with eculizumab-containing serum significantly decreased cell killing compared with HELLP serum alone.
The kill rate in women with classic or atypical HELLP decreased from 34% to 5% with eculizumab.
Eculizumab (Soliris), manufactured by Alexion Pharmaceuticals, is a monoclonal antibody approved by the US Food and Drug Administration for the treatment of paroxysmal nocturnal hemoglobinuria (PNH) and aHUS.
Further investigation is required to confirm these findings, but thus far, the investigators believe the modified Ham assay may assist in diagnosing the HELLP syndrome and corroborate its relationship to aHUS.
The current study by Vaught et al is published in Experimental Hematology. 
Photo by Nina Matthews
A blood test developed to diagnose a rare genetic blood cell disorder, atypical hemolytic uremic syndrome (aHUS), may also aid in the diagnosis of HELLP syndrome, a life-threatening high blood pressure condition that affects 1% of all pregnant women.
The study, based on blood samples from a small number of women, suggests that aHUS has similar underlying biochemistry to HELLP, which affects hemolysis, elevates liver enzymes, and causes a low platelet count. Both conditions have over activation of the alternative pathway of complement.
At present, no diagnostic blood or biomarker test exists to diagnose HELLP, which is thought to be a severe form of preeclampsia. The condition is diagnosed only by its symptoms.
Senior study author Robert Brodsky, MD, of Johns Hopkins, and his team developed the modified Ham test to diagnose aHUS, a genetic disorder in which abnormal blood clots form in small blood vessels in the kidneys. They published that work last year in Blood.
The two conditions share a number of traits, such as hemolysis, elevated liver enzymes, a low platelet count, kidney dysfunction, high blood pressure, and seizures. This led the investigators to believe that the modified Ham test could also help identify women with HELLP syndrome.
"The clinical implications from an obstetric point of view are potentially huge," said lead study author Arthur Vaught, MD, also of Johns Hopkins. "If this works, we can reduce pre-term deliveries, stays in the neonatal intensive care unit, and other complications for mothers and their babies."
The team analyzed serum samples from 14 women with classic or atypical HELLP syndrome, 7 with severe preeclampsia, 11 women with normal pregnancies, and 8 healthy nonpregnant women. All pregnant women were at least 23 weeks’ gestation, the point at which HELLP symptoms start to arise.
The team evaluated patient sera using terminal product of complement activation (C5b-9). They observed that women with classic or atypical HELLP had increased complement activation compared to nonpregnant controls.
Women with classic HELLP had an average cell killing of 34.3% compared with 26% in women with atypical HELLP, 5% in women with normal pregnancies, and3.3% in women who were not pregnant.
The investigators then added eculizumab to HELLP sera to see whether the agent could inhibit complement activation. They found that mixing HELLP serum with eculizumab-containing serum significantly decreased cell killing compared with HELLP serum alone.
The kill rate in women with classic or atypical HELLP decreased from 34% to 5% with eculizumab.
Eculizumab (Soliris), manufactured by Alexion Pharmaceuticals, is a monoclonal antibody approved by the US Food and Drug Administration for the treatment of paroxysmal nocturnal hemoglobinuria (PNH) and aHUS.
Further investigation is required to confirm these findings, but thus far, the investigators believe the modified Ham assay may assist in diagnosing the HELLP syndrome and corroborate its relationship to aHUS.
The current study by Vaught et al is published in Experimental Hematology.
Photo by Nina Matthews
A blood test developed to diagnose a rare genetic blood cell disorder, atypical hemolytic uremic syndrome (aHUS), may also aid in the diagnosis of HELLP syndrome, a life-threatening high blood pressure condition that affects 1% of all pregnant women.
The study, based on blood samples from a small number of women, suggests that aHUS has similar underlying biochemistry to HELLP, which affects hemolysis, elevates liver enzymes, and causes a low platelet count. Both conditions have over activation of the alternative pathway of complement.
At present, no diagnostic blood or biomarker test exists to diagnose HELLP, which is thought to be a severe form of preeclampsia. The condition is diagnosed only by its symptoms.
Senior study author Robert Brodsky, MD, of Johns Hopkins, and his team developed the modified Ham test to diagnose aHUS, a genetic disorder in which abnormal blood clots form in small blood vessels in the kidneys. They published that work last year in Blood.
The two conditions share a number of traits, such as hemolysis, elevated liver enzymes, a low platelet count, kidney dysfunction, high blood pressure, and seizures. This led the investigators to believe that the modified Ham test could also help identify women with HELLP syndrome.
"The clinical implications from an obstetric point of view are potentially huge," said lead study author Arthur Vaught, MD, also of Johns Hopkins. "If this works, we can reduce pre-term deliveries, stays in the neonatal intensive care unit, and other complications for mothers and their babies."
The team analyzed serum samples from 14 women with classic or atypical HELLP syndrome, 7 with severe preeclampsia, 11 women with normal pregnancies, and 8 healthy nonpregnant women. All pregnant women were at least 23 weeks’ gestation, the point at which HELLP symptoms start to arise.
The team evaluated patient sera using terminal product of complement activation (C5b-9). They observed that women with classic or atypical HELLP had increased complement activation compared to nonpregnant controls.
Women with classic HELLP had an average cell killing of 34.3% compared with 26% in women with atypical HELLP, 5% in women with normal pregnancies, and3.3% in women who were not pregnant.
The investigators then added eculizumab to HELLP sera to see whether the agent could inhibit complement activation. They found that mixing HELLP serum with eculizumab-containing serum significantly decreased cell killing compared with HELLP serum alone.
The kill rate in women with classic or atypical HELLP decreased from 34% to 5% with eculizumab.
Eculizumab (Soliris), manufactured by Alexion Pharmaceuticals, is a monoclonal antibody approved by the US Food and Drug Administration for the treatment of paroxysmal nocturnal hemoglobinuria (PNH) and aHUS.
Further investigation is required to confirm these findings, but thus far, the investigators believe the modified Ham assay may assist in diagnosing the HELLP syndrome and corroborate its relationship to aHUS.
The current study by Vaught et al is published in Experimental Hematology.