CHRONIC LYMPHOCYTIC LEUKAEMIA CLL
CLL is the most common leukemia in Westren countries, accounting for one-third of cases. The disease is rare in Asians; 90% of patients are older than 50, median age at presentation is between 65 and 70 years Men are affected more often than women by a ratio of 2:1.PATHOOGY AND NATURAL HISTORY
PATHOLOGY; CLL RESULTS FROM SUPPRESION OF PROGRAMMED CELL DEATH(APOPTOSIS) OF MATURE B CELL. SURFACE MEMBRANE ANTIGENS INCLUDE THE B CELL ANTIGENS CD 19, CD 20, CD23 . CD5 IS ALWAYS PRESENT ON CLL CELLS. CD38 HAS BEEN ASSOCIATED WITH UNFAVORABLE PROGNOSISNATURAL HISTORY
1. Immunological abnrmalities; a. Advanced disease is associated with hypogammaglobulinemia and decreased humoral respnses to antigens. b. Avariety of in vitro lymphocyte function test are abnormal. c. Coomb`s postive warm antibody hemolytic anemia occurs in 10% & immune thrombocytopenia in about 5%.2. Clinical course
*Survival is closely correlated with the stage of disease at time of diagnosis. *Because most of patients are elderly at time of diagnosis ; more than 30% die of diseases untrelated to leukemia. A. Manifestation. In 70% of patients CLL is first recognized at routine physical exam.or by routine CBC. Clinical manifestation develop as the leukemic cell acumalate on lymph nodes ,liver ,spleen & bone marrow . Presenting problems may be anaemia, infections,painless lymphadenopathy, and systemic symptoms such as night sweats or weight loss. However, these more often occur later in the course of the disease.Clinical course-Cont.
Transformation in to a diffuse large B-cell lymphoma(Richter`s syndrome) or prolymphocytic leukemia occurs in less than 5% of patients. b. progressive disease *Death is usually due to infection , bleeding or other complicatin of the disease. 1.HERPES ZOSTER is the cause of 10% infection in CLL. 2.Bacterial pathogens associated with hypogammoglobuliemia include Streptococus pneumoniae ,Staphylococcus auerus and Hemophilus influenzae . 3.Pneumocystis jiroveciiLaboratory studies
1. Hemogram a.Erythrcytes; anemia may be caused by 1.bone marrow infiltration. 2.hypersplenism 3.autoimmune hemolysis b. Lymphocytes; the absolute count ranges from; 10x109/l - 200x109/l but may exceed 500x109/l. *When blod smears are made , the cells are easily ruptured , producing typical “basket” or “smudge” cells.c . Granulocyte; absolute counts are normal or increased until late in the disease.d .Platelets; thrombocytopenia may prodused by bone marrow infiltration , hypersplenism, immune thrombocytopenia‘smudge’ cells lymphocytes
CHRONIC LYMPHOCYTIC LEUKEMIA
*DIAGNOSIS 1. Lymphocytes ( x109/L) > 5; > 1 B-cell marker (CD 19, CD20 , CD 23) + CD5 2. Atypical cells (prolymphocyte) (%) < 55 3. Bone marrow lymphocytes ( %) >30Binet Staging System
*Area of involvement considered for staging. 1. Head & neck , including the Waldeyer ring ( this counts as one area even if more than one group of nodes are enlarged ) 2. Axillae ( involvement of both axillae count as one area) 3. Groins, including superficial femorals counts as one area.Binet Staging System
Stage A. Hb > 100 g /L & platelets > 100 x109/L & up to two of the above lymph node involved. Stage B. Hb > 100 g / L & platelets > 100 x109 / L & three or more areas of nodal or organ enlargement. Stage C. All patients , irrespective of organomegaly in whom Hb < 100 g / L & or platelets < 100 x 109 / L.Indications for Therapy in B cell- CLL
Anemia Thrombocytopenia Disease- related symptoms Markedly enlarged or painful spleen symptomatic lymphadenopathy Blood lymphocyte count doubling time < 6 months Prolymphocytic transformation Richter`s transformationRESPONSE CRITERIA
Complete remission(CR)PARTIAL REMISSION(PR)
TREATMENT
**Prolymphocytic leukaemia**Hairy cell leukaemia