Pathology
Haematology
Question 39 of 180
Regarding Hodgkin lymphoma, which of the following statements is CORRECT:
Answer:
Lymphomas are a group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and other lymphoid tissue and cause the characteristic clinical feature of lymphadenopathy. Hodgkin lymphoma can present at any age but is rare in children and has a peak incidence in young adults. There is an almost 2 : 1 male predominance. Most patients present with painless, asymmetrical, firm and discrete enlargement of superficial lymph nodes. Cervical nodes are involved in 60-70% of cases, axillary nodes in 10-15% and inguinal nodes in 6-12%. Modest splenomegaly occurs during the course of the disease in 50% of patients; the liver may also be enlarged. Bone marrow failure is unusual in early disease.Lymphoma
Pathology / Haematology / Malignancy
Last Updated: 25th April 2019
Lymphomas are a group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and other lymphoid tissue and cause the characteristic clinical feature of lymphadenopathy.
The major subdivision of lymphomas is into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) and this is based on the histological presence of Reed-Sternberg cells present in HL.
Hodgkin Lymphoma
Incidence:
Hodgkin lymphoma can present at any age but is rare in children and has a peak incidence in young adults. There is an almost 2 : 1 male predominance.
Clinical Features:
- Lymphadenopathy
- Most patients present with painless, asymmetrical, firm and discrete enlargement of superficial lymph nodes
- Cervical nodes are involved in 60-70% of cases, axillary nodes in 10-15% and inguinal nodes in 6-12%
- Typically the disease is localised initially to a single peripheral lymph node region and its subsequent progression is by contiguity within the lymphatic system
- Retroperitoneal nodes are also often involved but usually only diagnosed by CT scan
- Modest splenomegaly occurs during the course of the disease in 50% of patients; the liver may also be enlarged
- Mediastinal involvement is found in up to 10% of patients at presentation (may be associated with pleural effusions or superior vena cava obstruction)
- Cutaneous HL occurs as a late complication in approximately 10% of patients
- Constitutional symptoms are prominent in patients with widespread disease
- Fever (occurs in 30% of patients)
- Pruritus (occurs in 25% of patients)
- Alcohol-induced pain in areas where disease is present
- Other symptoms including weight loss, night sweats, fatigue, weakness, anorexia and cachexia
Investigations:
- Normochromic normocytic anaemia is most common; bone marrow failure involvement is unusual in early disease, but if it occurs bone marrow failure may develop with leucoerythroblastic anaemia
- One-third of patients have a neutrophilia; eosinophilia is frequent
- Advanced disease is associated with lymphopenia and loss of cell-mediated immunity
- Platelet count is normal or increased in early disease and reduced in later stages
- ESR and CRP are usually raised (ESR is useful in monitoring disease progress)
- Serum LDH is raised initially in 30-40% of cases
- Diagnosis is made by histological examination of an excised lymph node
- The distinctive multinucleate polypoid RS cell is central to the diagnosis of the four classic types of HL (95% of cases)
Management:
- The selection of appropriate treatment depends on the stage and grade of the disease
- Staging is performed by history, clinical examination and blood tests together with PET and CT scans
- Treatment is with chemotherapy alone or a combination of chemotherapy with radiotherapy; the response to treatment can be monitored by CT and PET scans; disease relapse can be treated with chemotherapy and sometimes with stem cell transplantation
Prognosis:
- The prognosis depends on age, stage and histology
- Overall approximately 85% of patients are cured
Non-Hodgkin Lymphoma
Non-Hodgkin lymphomas (NHL) are a large group of clonal lymphoid tumours, about 85% of B-cell and 15% of T or NK (natural killer) cell origin. Their clinical presentation and natural history are much more variable than Hodgkin lymphoma. They are characterised by an irregular pattern of spread and a significant proportion of patients develop disease outside of the lymph nodes.
Clinical features:
- Superficial lymphadenopathy (typically asymmetric and painless)
- Constitutional symptoms (fever, night sweats, weight loss)
- Oropharyngeal involvement (sore throat, noisy or obstructed breathing)
- Symptoms due to anaemia, neutropaenia or thrombocytopaenia
- Abdominal disease (hepatosplenomegaly, involvement of retroperitoneal or mesenteric nodes)
- Infiltration of other organs (skin, brain, testis or thyroid)
Investigations:
- Whole lymph node biopsy, or more usually trucut biopsy of lymph node or of other involved tissue (e.g. bone marrow or extranodal tissue) is the definitive investigation; morphological examination of the biopsy is assisted by immunophenotypic, and in some cases, genetic analysis
- Blood tests
- PET/CT imaging
Management:
- When treatment is started it is usually in the form of a combination of chemotherapy drugs together with a monoclonal antibody directed against the tumour cell
- However, various new drugs have been developed which may change the management of disease in future years
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- Biochemistry
- Blood Gases
- Haematology
| Biochemistry | Normal Value |
|---|---|
| Sodium | 135 – 145 mmol/l |
| Potassium | 3.0 – 4.5 mmol/l |
| Urea | 2.5 – 7.5 mmol/l |
| Glucose | 3.5 – 5.0 mmol/l |
| Creatinine | 35 – 135 μmol/l |
| Alanine Aminotransferase (ALT) | 5 – 35 U/l |
| Gamma-glutamyl Transferase (GGT) | < 65 U/l |
| Alkaline Phosphatase (ALP) | 30 – 135 U/l |
| Aspartate Aminotransferase (AST) | < 40 U/l |
| Total Protein | 60 – 80 g/l |
| Albumin | 35 – 50 g/l |
| Globulin | 2.4 – 3.5 g/dl |
| Amylase | < 70 U/l |
| Total Bilirubin | 3 – 17 μmol/l |
| Calcium | 2.1 – 2.5 mmol/l |
| Chloride | 95 – 105 mmol/l |
| Phosphate | 0.8 – 1.4 mmol/l |
| Haematology | Normal Value |
|---|---|
| Haemoglobin | 11.5 – 16.6 g/dl |
| White Blood Cells | 4.0 – 11.0 x 109/l |
| Platelets | 150 – 450 x 109/l |
| MCV | 80 – 96 fl |
| MCHC | 32 – 36 g/dl |
| Neutrophils | 2.0 – 7.5 x 109/l |
| Lymphocytes | 1.5 – 4.0 x 109/l |
| Monocytes | 0.3 – 1.0 x 109/l |
| Eosinophils | 0.1 – 0.5 x 109/l |
| Basophils | < 0.2 x 109/l |
| Reticulocytes | < 2% |
| Haematocrit | 0.35 – 0.49 |
| Red Cell Distribution Width | 11 – 15% |
| Blood Gases | Normal Value |
|---|---|
| pH | 7.35 – 7.45 |
| pO2 | 11 – 14 kPa |
| pCO2 | 4.5 – 6.0 kPa |
| Base Excess | -2 – +2 mmol/l |
| Bicarbonate | 24 – 30 mmol/l |
| Lactate | < 2 mmol/l |
