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Case study (43) - Chronic Lymphocytic Leukemia (CLL)


Case study (43) - Chronic Lymphocytic Leukemia (CLL) 


A 68-year-old man showed a 12-hour history of headaches, confusion, and decreased consciousness. 

His wife said that he recently completed oral chemotherapy for "indolent forms of leukemia." 

Examination reveals him to be responding to painful stimuli but not to verbal commands. 

He has bilateral axillary and inguinal lymphadenopathy. 

He is clinically jaundiced and anemic. 

His spleen is palpably enlarged. 

He has neck stiffness, generalized hyperreflexia, and bilateral upgoing plantar reflexes. 

The fundal examination is normal, and there are no focal neurological signs. 

Complete blood count shows: 


Hemoglobin (Hb) 7.5 g/dL 

White blood cells (WBC) 37 X 109/L (lymphocytes 86%) 

Platelets 26 X 109/L 

Questions: 


Q1. What abnormalities are seen on this patient’s blood film? 




Q2. Suggest a possible hematological diagnosis. 

Q3. What abnormalities are seen on the enhanced computed tomography (CT) scan? 




Q4. How should this patient be managed? 

Answers: 


A1. The film shows spherocytosis, polychromasia, thrombocytopenia, and lymphocytosis. 

A2. The ‘indolent form of leukemia’ in this case was chronic lymphocytic leukemia (CLL), which became complicated by autoimmune hemolytic anemia (AIHA). (This is a recognized association.) 

Direct anti-globulin test, reticulocyte count, lymphoid cell flow cytometric analysis, and bone marrow examination is required to confirm this. 

The flow cytometry analysis will show these cells to be positive for B-cell markers [CD19, 20, 21, and 23] and to have a low density of surface membrane immunoglobulin [SmIg] with a predominance of one light chain, either kappa or lambda. 

B-CLL cells also express aberrantly T cell marker CD5. 

A3. The scan shows a low attenuation area in the right parieto-occipital area. 

There is no evidence of midline shift. 

This is inconsistent with acute bleeding but suggests infection, inflammation, or infarction. 

A4. He requires urgent lumbar puncture. 

In this case, it showed intracellular Gram-positive rods, consistent with Listeria meningitis. 

A low platelet count indicates the possibility of disseminated intravascular coagulation (DIC), which usually complicates meningitis. 

He received intravenous treatment of ampicillin, chloramphenicol, and metronidazole. 

His AIHA responded to high-dose intravenous steroids 

(dexamethasone was chosen to reduce cerebral edema) and blood transfusion. 

Fludarabine is best avoided as its use has been associated with an increased risk of AIHA in CLL patients. 

Other immunosuppressive for the AIHA may include rituximab, which may be beneficial for CLL. 

Splenectomy will increase the risk of infection. 
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