SCREENING
FOR PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) IN EGYPTIAN CHILDREN WITH
APLASTIC ANEMIA
S. Rizk *,
I. Youssry **, I. Mansour *
Clinical
Pathology* and Pediatrics** Departments, Cairo University
Objective: To screen for PNH clone
in Egyptian aplastic anemia pediatric patients before initiation of any
specific therapy & to evaluate the clinical status of studied patients
3-6 months after initiation of immunosuppressive therapy.
Methods: We studied eleven
pediatric patients with newly diagnosed acquired aplastic anemia and we
followed them up clinically for 3-6 months after initiation of
immunosuppressive therapy. In addition to routine clinical &laboratory
evaluation, sucrose lysis test and staining of bone marrow section for CD59
were done to all subjects.
Results: All studied cases had
severe aplastic anemia (SAA) except one case was very severe aplastic
anemia (VSAA). Sucrose lysis test was negative in all studied cases.
Presence of PNH clone (as evident by loss of normal staining of hematopoietic
cells for CD 59 = CD 59-ve cells) was evident in four cases (36.4%) of our
studied cases. One case (25%) with PNH clone developed splenic vein
thrombosis. As regard the laboratory data WBC<2.8X103 /cmm and Retics ≥0.6% were the most
frequent factors associated with PNH clone ( found in all PNH cases (100%),
but only in (28.6%) and
(14.3%) respectively of non-PNHcases. Mortality rate was higher in non-PNH
cases (28.5%) compared to (25%) of PNH cases.
Conclusion: Immunohistochemical
staining of bone marrow sections is a sensitive tool to detect the
emergence of PNH clone in aplastic anemia patients. thrombotic
complications should be anticipated in cases with aplastic anemia having a
PNH clone.