THE LYMPHOMAS - Clinical features, Clinical features, Investigations

The lymphomas are neoplastic transformations of normal B or T cells which reside predominantly in lymphoid tissues. They are commoner than the leukaemias and are increasing in incidence for reasons that are unclear. The disease is classified on the basis of histological appearance into Hodgkin's and non-Hodgkin's lymphoma (NHL).

Hodgkin’s lymphoma

Hodgkin's lymphoma is primarily a disease of young adults. Previous infection with Epstein-Barr virus (EBV) is thought to play a role in pathogenesis in some patients.

Clinical features

Painless lymph node enlargement (often cervical nodes) is the most common presentation. These lymph nodes have a rubbery consistency on examination. There may be hepatosplenomegaly. Systemic ‘B' symptoms are fever, drenching night sweats and weight loss (>10% in previous 6 months). Other constitutional symptoms such as pruritus, fatigue, anorexia and alcohol-induced pain at the site of the enlarged lymph nodes also occur.

Investigations

■ Blood count may be normal or show a normochromic, normocytic anaemia.

■ The erythrocyte sedimentation rate (ESR) is usually raised and is an indicator of disease activity.

■ Liver biochemistry may be abnormal, with or without liver involvement.

■ Serum lactate dehydrogenase (LDH) if raised is an adverse prognostic marker.

■ Chest X-ray may show mediastinal widening from enlarged nodes.

■ Diagnosis is by lymph node biopsy and histological examination showing Reed-Sternberg cells (binucleate or multinucleate malignant B lym-phocytes) in a background rich in benign small lymphocytes and histiocytes.

■ Disease staging is by computed tomography (CT) scan, which may show intrathoracic, abdominal and pelvic nodes. Positron emission tomography (PET) (p. 828) is increasingly being used for staging, assessment of response and direction of treatment.

Differential diagnosis

This includes any other cause of lymphadenopathy (Table 6.3). Persistently enlarged lymph nodes must always be excised for histological and micro-biological ex

Table 6.3 Differential diagnosis of lymphadenopathy
Localized Generalized
Pyogenic infection, e.g. tonsillitis
Tuberculosis
Epstein–Barr virus
Cytomegalovirus
Toxoplasma sp.
Tuberculosis
HIV infection
Lymphoma Lymphoma
Secondary carcinoma Leukaemia
  Systemic disease
Systemic lupus erythematosus
Sarcoidosis
Rheumatoid arthritis
  Drug reaction, e.g. phenytoin
HIV, human immunodeficiency virus.

Management

Treatment is given with curative intent. The choice of treatment depends on:

■ Stage (Table 6.4)

■ Involved sites

■ ‘Bulk' of lymph nodes involved

■ Presence of ‘B' symptoms.

■ ‘Early’ stage disease (Stage IA, IIA with no bulk) is treated with brief chemotherapy (ABVD, doxorubicin, bleomycin, vinblastine, dacarbazine) followed by involved field irradiation.

■ Advanced disease (all other stages) are treated with cyclical combination chemotherapy (8 cycles of ABVD) with irradiation at sites of bulk disease. PET/CT is used to detect disease activity after treatment and to distin-guish between active tumour (PET-positive) and necrosis or fibrosis (PET-negative) in residual masses. Irradiation, with its attendant complications, can be omitted in PET-negative masses after chemotherapy.

Prognosis is related to the stage of disease at presentation, with a 5-year survival rate of over 90% in stage I declining to 60% in stage IV. The presence of B symptoms indicates more severe disease with a worse prognosis.

Non-Hodgkin’s lymphoma

These are malignant tumours of the lymphoid system classified separately from Hodgkin's lymphoma. Most (70%) are of B cell origin with 30% of T cell

Table 6.4 Staging classification of Hodgkin's lymphoma

Stage

Definition

I Involvement of a single lymph node region or a single extralymphatic organ or site

II

Involvement of two or more lymph node regions on the same side of the diaphragm, or localized involvement of an extralymphatic organ or site and of one or more lymph node regions on the same side of the diaphragm

III1

Involvement of lymph node regions on both sides of the diaphragm, which may also be accompanied by involvement of the spleen (IIIS) or by localized involvement of an extralymphatic organ (IIIE) or site or both (IIISE).

IV

Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement

Each stage can also be graded A (no ‘B’ symptoms) or B (with ‘B’ symptoms), X (bulky
disease) or E (involvement of a single extranodal site that is contiguous or proximal to
the known nodal site).

origin. There is a malignant clonal expansion of lymphocytes, which occurs at different stages of lymphocyte development. In general, neoplasms of non-dividing mature lymphocytes are indolent whereas those of proliferating cells (e.g. lymphoblasts, immunoblasts) are much more aggressive. The B and T/natural killer (NK) cell lymphomas are each further divided on this basis, e.g. precursor B cell lymphoma, and then again subdivided based on cytogenetics and immunophenotyping. The aetiology is unknown in most cases but some are associated with a specific infection, e.g. Helicobacter pylori and gastric mucosa-associated lymphoid tissue (MALT) lymphoma.

Clinical features

Presentation is rare before the age of 40. Most patients present with painless peripheral lymph node enlargement. Systemic symptoms as in Hodgkin's lymphoma may occur. Extranodal involvement is more common that in Hodg-kin's lymphoma and almost any organ in the body can be involved. Bone marrow infiltration leads to anaemia, recurrent infections and bleeding. Skin involvement with T cell lymphoma presents as mycosis fungoides and Sézary syndrome.

Investigations

■ Blood count may show anaemia. An elevated white cell count or throm-bocytopenia suggests bone marrow involvement. The ESR may be raised.

■ Liver biochemistry may be abnormal if the liver is involved.

■ Serum lactate dehydrogenase and p2-microglobulin are prognostic indicators.

■ Chest X-ray, CT, PET and gallium scans are of help in staging.

■ Bone marrow aspiration and trephine biopsy will confirm marrow involvement.

■ Lymph node biopsy is required for definitive diagnosis and subtype classification.

Management

Treatment depends on the lymphoma type and stage (similar to Hodgkin's lymphoma). Diffuse large B cell lymphoma is the commonest lymphoma and first-line treatment is with cyclical combination chemo-immunotherapy (CHOP + R, cyclophosphamide, hydroxydaunorubicin, vincristine, pred-nisolone and rituximab) with field irradiation for those with bulky disease. Between 60-70% of those with early-stage disease will achieve a cure with this regimen.

■ Primary gastric lymphoma is many cases is associated with H. pylori infection. Treatment to eradicate the infection (p. 80) is usually all that is required provided there is no evidence of disease outside the stomach. This is followed by close endoscopic surveillance.

■ Burkitts lymphoma occurs mainly in African children, and is associated with EBV infection. Jaw tumours are common, usually with gastrointesti-nal involvement. Treatment is with cyclical combination chemotherapy.

Ebook Essentials of Kumar and Clark's Clinical Medicine, 5e

1. Ethics and communication

Ethics and communication

2. Infectious diseases

Infectious diseases

3. Gastroenterology and nutrition

Gastroenterology and nutrition

4. Liver, biliary tract and pancreatic disease

Liver, biliary tract and pancreatic disease
LIVER BIOCHEMISTRY AND LIVER FUNCTION TESTS
SYMPTOMS AND SIGNS OF LIVER DISEASE
JAUNDICE
HEPATITIS
NON - ALCOHOLIC FATTY LIVER DISEASE (NAFLD)
CIRRHOSIS
COMPLICATIONS AND EFFECTS OF CIRRHOSIS
LIVER TRANSPLANTATION
TYPES OF CHRONIC LIVER DISEASE AND CIRRHOSIS
PRIMARY SCLEROSING CHOLANGITIS
BUDD - CHIARI SYNDROME
LIVER ABSCESS
LIVER DISEASE IN PREGNANCY
LIVER TUMOURS
GALLSTONES
THE PANCREAS
CARCINOMA OF THE PANCREAS
NEUROENDOCRINE TUMOURS OF THE PANCREAS

5. Haematological disease

Haematological disease
ANAEMIA
Assessment and treatment of suspected neutropenic sepsis
HAEMOLYTIC ANAEMIA
INHERITED HAEMOLYTIC ANAEMIAS
ACQUIRED HAEMOLYTIC ANAEMIA
MYELOPROLIFERATIVE DISORDERS
THE SPLEEN
BLOOD TRANSFUSION
THE WHITE CELL
HAEMOSTASIS AND THROMBOSIS
THROMBOSIS
THERAPEUTICS

6. Malignant disease

Malignant disease
MYELOABLATIVE THERAPY AND HAEMOPOIETIC STEM CELL TRANSPLANTATION
THE LYMPHOMAS
THE PARAPROTEINAEMIAS
PALLIATIVE MEDICINE AND SYMPTOM CONTROL

7. Rheumatology

Rheumatology
COMMON INVESTIGATIONS IN MUSCULOSKELETAL DISEASE
COMMON REGIONAL MUSCULOSKELETAL PROBLEMS
BACK PAIN
OSTEOARTHRITIS
INFLAMMATORY ARTHRITIS
THE SERONEGATIVE SPONDYLOARTHROPATHIES
Clinical features, Investigations
INFECTION OF JOINTS AND BONES
AUTOIMMUNE RHEUMATIC DISEASES
SYSTEMIC INFLAMMATORY VASCULITIS
DISEASES OF BONE
THERAPEUTICS

8. Water, electrolytes and acid–base balance

WATER AND ELECTROLYTE REQUIREMENTS
BODY FLUID COMPARTMENTS
REGULATION OF BODY FLUID HOMEOSTASIS
PLASMA OSMOLALITY AND DISORDERS OF SODIUM REGULATION
DISORDERS OF POTASSIUM REGULATION
DISORDERS OF MAGNESIUM REGULATION
DISORDERS OF ACID - BASE BALANCE
THERAPEUTICS

9. Renal disease

Renal disease
INVESTIGATION OF RENAL DISEASE
GLOMERULAR DISEASES
NEPHROTIC SYNDROME
URINARY TRACT INFECTION
TUBULOINTERSTITIAL NEPHRITIS
HYPERTENSION AND THE KIDNEY
RENAL CALCULI AND NEPHROCALCINOSIS
URINARY TRACT OBSTRUCTION
ACUTE RENAL FAILURE/ACUTE KIDNEY INJURY
CHRONIC KIDNEY DISEASE
RENAL REPLACEMENT THERAPY
CYSTIC RENAL DISEASE
TUMOURS OF THE KIDNEY AND GENITOURINARY TRACT
DISEASES OF THE PROSTATE GLAND
TESTICULAR TUMOUR
URINARY INCONTINENCE

10. Cardiovascular disease

COMMON PRESENTING SYMPTOMS OF HEART DISEASE
INVESTIGATIONS IN CARDIAC DISEASE
CARDIAC ARRHYTHMIAS
HEART FAILURE
ISCHAEMIC HEART DISEASE
RHEUMATIC FEVER
VALVULAR HEART DISEASE
PULMONARY HEART DISEASE
MYOCARDIAL DISEASE
CARDIOMYOPATHY
PERICARDIAL DISEASE
SYSTEMIC HYPERTENSION
ARTERIAL AND VENOUS DISEASE
ELECTRICAL CARDIOVERSION
DRUGS FOR ARRHYTHMIAS
DRUGS FOR HEART FAILURE
DRUGS AFFECTING THE RENIN - ANGIOTENSIN SYSTEM
NITRATES, CALCIUM - CHANNEL BLOCKERS AND POTASSIUM - CHANNEL ACTIVATORS

11. Respiratory disease


Respiratory disease
TUBERCULOSISnd
DIFFUSE DISEASES OF THE LUNG PARENCHYMA
OCCUPATIONAL LUNG DISEASE
CARCINOMA OF THE LUNG
DISEASES OF THE CHEST WALL AND PLEURA
DISORDERS OF THE DIAPHRAGM

12. Intensive care medicine

Intensive care medicine

13. Drug therapy, poisoning, and alcohol misuse

Drug therapy, poisoning, and alcohol misuse

14. Endocrine disease

Endocrine disease
PITUITARY HYPERSECRETION SYNDROMES
THE THYROID AXIS
MALE REPRODUCTION AND SEX
FEMALE REPRODUCTION AND SEX
THE GLUCOCORTICOID AXIS
THE THIRST AXIS
DISORDERS OF CALCIUM METABOLISM
DISORDERS OF PHOSPHATE CONCENTRATION
ENDOCRINOLOGY OF BLOOD PRESSURE CONTROL
DISORDERS OF TEMPERATURE REGULATION
THERAPEUTICS

15. Diabetes mellitus and other disorders of metabolism

DIABETES MELLITUS
DIABETIC METABOLIC EMERGENCIES
COMPLICATIONS OF DIABETES
SPECIAL SITUATIONS
HYPOGLYCAEMIA IN THE NON - DIABETIC
DISORDERS OF LIPID METABOLISM
THE PORPHYRIAS

16. The special senses

THE EAR
THE NOSE AND NASAL CAVITY
THE THROAT
THE EYE

17. Neurology

COMMON NEUROLOGICAL SYMPTOMS
COORDINATION OF MOVEMENT
THE CRANIAL NERVES
COMMON INVESTIGATIONS IN NEUROLOGICAL DISEASE
UNCONSCIOUSNESS AND COMA
STROKE AND CEREBROVASCULAR DISEASE
EPILEPSY AND LOSS OF CONSCIOUSNESS
NERVOUS SYSTEM INFECTION AND INFLAMMATION
HYDROCEPHALUS
HEADACHE, MIGRAINE AND FACIAL PAIN
SPINAL CORD DISEASE
DEGENERATIVE NEURONAL DISEASES
DISEASES OF THE PERIPHERAL NERVES
MUSCLE DISEASES
MYOTONIAS
DELIRIUM
THERAPEUTICS

18. Dermatology

Dermatology

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