Case 4: A 76-year-old woman presented with a 6-month history of weakness, malais
ID: 60430 • Letter: C
Question
Case 4: A 76-year-old woman presented with a 6-month history of weakness, malaise, exertional dyspnea and abdominal discomfort. In the previous month she had experienced two dizzy spells but did not have headaches, visual disturbances, weight loss, bone pain or recurrent infections. On examination she was pale, with moderate axillary and cervical lymphadenopathy. Her liver and spleen were enlarged by 5cm.
Test
Patient
Reference
Test
Patient
Reference
Hemoglobin
10.8 g/dL
Serum Protein
13.0 g/dL
WBC
8.2/uL
IFE
IgM, kappa
Differential
Normal
IgG
.94 mg/dL
ESR
112 mm/h
IgA
.11 mg/dL
IgM
6.45 mg/dL
IgM paraprotein (M band)
6.3 mg/dL
Electrophoresis of concentrated urine showed no free monoclonal light chains and there were no bone lesions on X-rays of her chest and skull. Her serum viscosity, relative to water, was 4.7 (NR 1.4-1.8). A bone marrow examination showed a pleomorphic cellular infiltrate composed of a mixture of small lymphocytes, plasma cells and cells of an intermediate appearance, called lymphoplasmacytoid cells.
1.From the above data, what disease should the doctor suspect?
2.Why did the urine show no free monoclonal light chains?
3.Why does the patient show Axillary and cervical lymphadenopathy?
4.Why were X-rays done of her chest and skull?
5.What is the prognosis for this disease?
Test
Patient
Reference
Test
Patient
Reference
Hemoglobin
10.8 g/dL
Serum Protein
13.0 g/dL
WBC
8.2/uL
IFE
IgM, kappa
Differential
Normal
IgG
.94 mg/dL
ESR
112 mm/h
IgA
.11 mg/dL
IgM
6.45 mg/dL
IgM paraprotein (M band)
6.3 mg/dL
Explanation / Answer
1. The doctor can suspect it as chronic lymphocytic leukaemia
2. These patients are difficult to diagnose as they frequently show no abnormality when tested by Serum Protein Electrophoresis (SPE). For this reason it has historically been recommended that a 24 hour urine collection is tested for the presence of monoclonal free light chains. However, urine tests for free light chains can be negative early in the disease as the capacity for reabsorption of light chains by the kidneys must be exceeded before free light chains appear in the urine.
3.
4. to check for the severity of the disese
5. prognosis:
Stage
A lower stage at the time of diagnosis is a more favourable prognostic factor.
Bone marrow involvement
A diffuse (more spread out) pattern of abnormal lymphocytes (leukemia cells) in a bone marrow sample is a less favourable prognostic factor. Nodular (small clumps) or interstitial (placed between cells) patterns of abnormal lymphocytes in a bone marrow sample are more favourable prognostic factors.
Age
Elderly people have a less favourable prognosis. Older age may be associated with a poorer performance statusperformance statusThe measure of how well a person is able to perform ordinary tasks and carry out daily activities. and other health problems that can affect how well people tolerate treatment.
Sex
Men have a less favourable prognosis than women.
Chromosome changes
Deletion of part of chromosome 13, with no other chromosome abnormalities, is a more favourable prognostic factor. Deletion of parts of chromosome 11 or 17 is a less favourable prognostic factor.
Proportion of lymphocytes
A higher number of abnormal lymphocytes in the blood gives a less favourable prognosis.
Doubling time of lymphocytes
Lymphocyte doubling time (the time it takes for the lymphocyte count to double) of less than 12 months is a less favourable prognostic factor.
Serum lactate dehydrogenase blood level
A high level of serum lactate dehydrogenaselactate dehydrogenaseAn enzyme that is involved in energy production in cells. (LDH) in the blood is a less favourable prognostic factor. This can be caused by increased production of lymphocytes or death of leukemia cells.
Blood protein levels
A high blood level of beta-2-microglobulin is a less favourable prognostic factor.
Fewer lymphocytes that have proteins like CD38 (cluster of differentiation 38) or ZAP-70 (zeta-associated protein 70) is a more favourable prognostic factor.
Richter's syndrome
Richter's syndrome, or a Richter transformation, occurs when CLL develops into an aggressive non-Hodgkin lymphoma, usually a diffuse large B-cell lymphoma. Richter's syndrome is a less favourable prognostic factor.
Performance status
People with a low performance status at the time of diagnosis have a less favourable prognosis. This may be due to existing health concerns that make them less tolerant to treatment.
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