الفهرس 
B-CELL chrONIC LYMPHOPROLIFERATIVE DISORDERSOnly 14 pages are availabe for public view
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Abstract
The B-cell chronic lymphoproliferative disorders are a T heterogeneous group of B-cell malignancy of clonal origin with a highly variable clinical course. They are morphologically, immunologically and clinically heterogeneous. They refer to several conditions in which lymphocytes are produced in excessive quantities. The accurate discrimination of each separate B-CLPD is of paramount importance because the prognosis and treatment differ widely for the different types.
The commonest B-CLPD is CLL, which characteristically expresses CD5 and CD19 on the cell surface. The differential diagnosis of a CD5/CD19 dual positive LPD lies mainly between CLL and MCL. MCL has a significantly poorer prognosis than CLL, often requiring more aggressive treatment; thus it is of clinical importance to discriminate between them.
CD1d is a non-polymorphic HLA class I–like, B2M –associated glycoprotein that present lipids and glycolipids at the cell surface for recognition by T lymphocytes. CD1d is expressed widely in normal hematopoietic and non hematopoietic cells such as thymocytes, monocytes, macrophages, primitive hematopoietic stem cells, keratinocytes, and hepatocytes, whereas normal peripheral blood B cells exhibit constitutive expression of CD1d. As regards hematopoietic malignancies, myeloid and lymphoid acute leukemias variably express CD1d.
This work aims to evaluate the diagnostic usefulness of CD1d in diagnosis of CLL and its value in differentiation of CLL from other B-CLPDs.
The present study was conducted on forty three adult patients diagnosed as B-CLBDs; they were classified into twenty patients with CLL, ten patients with MCL and thirteen patients with other B-CLPDs including HCL, SMZL and FL who attended Ain Shams University Hospitals to be diagnosed in the clinical and chemical pathology department –Ain Shams University. As well as ten age and sex matched healthy subjects as a control group to evaluate CD1d expression on normal B-lymphocytes.
Clinically, no statistical significant difference was detected between CLL and MCL cases and between B-NHL and MCL cases regarding; the presence of hepatomegaly and splenomegaly, however a statistical significant difference was observed regarding the presence of LN mainly in cases of MCL. Whereas no statistical significant difference between CLL and B-NHL cases was detected regarding; lymphadenopathy, hepatomegaly and splenomegaly.As regards routine laboratory data of patients, no statistical significant difference was detected between CLL and MCL cases and between CLL and B-NHL cases regarding TLC, ALC and platelet count, but as regards Hb concentration, statistical significant difference was observed. Whereas no statistical significant difference was detected between B-NHL and MCL cases regarding TLC, ALC, Hb level and platelet count.
We investigated the expression of CD1d on B cell of normal control group and on neoplastic B cells of patients with CLL, MCL and other B-NHL.
Using ROC curve to assess the best cut off point with a sensitivity and specificity for the diagnosis of CLL and the differentiation of CLL from MCL and other B-NHL; the present study revealed that:
The best cut-off value of CD1d% expression on B cells between CLL and control was <4.96%, where it had a diagnostic sensitivity of 100% and specificity of 100%, positive predictive value of 100% and negative predictive value of 100%.
The best cut-off value of CD1d% expression on B cells to differentiate between CLL and MCL was< 4.96%, where it had a diagnostic sensitivity of 100% and specificity of 90%, with positive predictive value and negative predictive value of 95.2% and 100% respectively.
Whereas, the best cut-off value of CD1d% expression on B cells between CLL and B-NHL was <3.5%, where it had diagnostic sensitivity of 95% and specificity of 76.92%, with positive predictive value and negative predictive value of 86.4% and 90.9% respectively
In addition, CD1d % expression was positively associated with CD38 expression among CLL group but as regards MFI, no statistical significant difference was found.
In conclusion, the present study, points to the importance of adding CD1d to the B-CLPDs flow cytometric routine panels as it revealed the presence of clear-cut between CLL cells, in which CD1d is expressed in lower levels compared with healthy control, B-NHL and MCL that showed high expression of CD1d. Our results also denoted a positive association between CD38 positivity and median CD1d% expression in CLL patients that might indicates a prognostic significance of CD1d expression that requires further investigations on the molecular level to prove its significance as a rapid, inexpensive and reliable prognostic marker. Therefore, adding CD1d in FCM routine panels could be of great help in distinguishing between B-CLPDs especially between CLL and MCL in particular in patients with a prevalent leukemic expression and inconclusive phenotypes.