Chronic Lymphocytic Leukaemia - Lymphoproliferative Disorders

Chronic Lymphocytic Leukaemia

·        Monoclonal proliferation of mature B cells (CD 19 & 20+). Being mature, will have surface expression of immunoglobulins

Epidemiology

·        Commonest leukaemia: 25%.

·        Primarily elderly

·        Male = 2 * Female

Clinical Features

·        Asymptomatic (40 – 70%)

·        Insidious, maybe weight loss, fatigue

·        Symmetrical enlargement of superficial lymph nodes (50%)

·        Splenomegaly and hepatomegaly 

·        ¯Platelets ® bruising 

·        Defects in CMI and ¯Ig ® infections: Herpes Zoster (shingles), fungal, bacterial, viral. Death usually due to infection

Diagnostic Criteria

·        Blood lymphocytes > 5 * 10E9/L

·        Lymphocytes are B cells (CD19, 20 and 24)

·        Marrow lymphocytosis > 30%

Differential Diagnosis

·        Reactive lymphocytosis: EBV, CMV, HZV, Toxoplasmosis, Brucellosis, Tb, Viral

·        Other B cell tumours:

o   Prolymphocytic anaemia

o   Hairy cell leukaemia

o   Splenic lymphoma with villous lymphocytes

o   Mantle cell lymphoma

o   Follicular lymphoma

Lab

·        20% diagnosed on routine blood test 

·        Lymphocytosis: > 5 * 10E9/L, but may be 30 to 300. Small lymphocytes and smudge cells common. (cytoplasm fragile – breaks easily)

·        Normal looking lymphocyte: small, little/no cytoplasm 

·        Anaemia in later stages due to marrow replacement and ¯survival. 15% have Combes positive haemolytic anaemia 

·        Marrow: lymphocytic replacement

·        10% have haemolytic anaemia

Treatment

·        Only if nodes painful: Prednisone (1 mg/kg), Chlorambucil, fludarabine. Side effects of prednisone: weight gain, hyperexia, mood changes (euphoria ® depression), candidiasis, polyuria (secondary to glucose intolerance), dyspepsia

·        Supportive treatment for infections, and radiotherapy for deposits causing pressure symptoms

·        Little impact on viscosity (its mainly RBCs and blasts that affect that)

·        Doesn‟t convert to Acute Lymphatic Leukaemia

·        Median survival from diagnosis: 4 years.  But 15% live for 15 years with no treatment

Complications Include

·        Infections, secondary to hypogammaglobulinaemia, neutropenia, drugs (immunosuppressive)

·        Cardia dysfunction: secondary to chemo toxicity, etc

·        DVT: hypercoagulable