The impact of the World Health Organization classification and clonality assessment of posttransplant lymphoproliferative disorders on disease management.
BACKGROUND
The World Health Organization classification of posttransplant lymphoproliferative disorders divides them into 4 main categories.


OBJECTIVE
To classify cases of posttransplant lymphoproliferative disorders diagnosed in our institution according to the World Health Organization scheme and correlate the classification and clonality with clinical data.


METHODS
Cases of posttransplant lymphoproliferative disorders were reviewed. They were classified according to the World Health Organization scheme. Clonality was determined by flow cytometry and/or polymerase chain reaction. Patients' charts were reviewed.


RESULTS
Thirty-one cases were identified. Median age was 33 years. There were 19 cases of kidney, 8 cases of liver, and 4 cases of bone marrow transplant. Immunosuppression consisted of cyclosporin A and prednisone (N = 24) or FK506 and prednisone (N = 7). Twenty cases (63%) were World Health Organization type 3, 7 cases (23%) type 2, 3 cases (6.4%) type 1, and 1 case type 4 posttransplant lymphoproliferative disorder. Ten patients received chemotherapy, 20 patients had reduction of immunosuppression, and 1 had no treatment. Follow-up was available on 25 patients. Seven (43.75%) of 16 with type 3 lesions with available follow-up died of their disease. Five of these patients received reduction of immunosuppression alone. Only 2 of 9 patients with type 3 disease who received chemotherapy died of disease. Two patients with type 2 disease died of unrelated causes. One patient is alive with disease; the remaining patients with types 1 and 2 disease are alive with no disease.


CONCLUSIONS
The World Health Organization classification of posttransplant lymphoproliferative disorders is valuable in the identification of subtypes. It helps identify early lesions (1 and 2) requiring reduction of immunosuppression and type 3 disease, which requires chemotherapy from the outset.
Mourad
Walid A
WA
King Faisal Specialist Hospital and Research Center, Department of Pathology, Riyadh, Saudi Arabia 11211. wmourad@hotmail.com
Tulabah
Asma
A
Al Sayed
Adher
A
Raja
Madras
M
Khafaga
Yasser
Y
El Gamal
Hazem
H
Al Shabanah
Mohamed
M
Al Meshari
Khaled
K
Al Shaibani
Khaled
K
Ezzat
Adnan
A
D000293:Adolescent; D000328:Adult; D000368:Aged; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005434:Flow Cytometry; D015321:Gene Rearrangement; D019672:Genes, T-Cell Receptor; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000145:classification* / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D016377:Organ Transplantation / Q000009:adverse effects*; D010641:Phenotype; D016896:Treatment Outcome; D014944:World Health Organization
D007143:Immunoglobulin Heavy Chains