The haematological system

Table 8.1)


Patients with anaemia may present with weakness, tiredness, dyspnoea, fatigue or postural dizziness. Anaemia due to iron deficiency is often the result of gastrointestinal blood loss, or sometimes recurrent heavy menstrual blood loss, and so these symptoms should be sought. Disorders of platelet function or blood clotting may present with easy-bruising or bleeding problems. Recurrent infection may be the first symptom of a disorder of the immune system, including leukaemia or HIV infection. The patient may have noticed lymph node enlargement, which can occur with lymphoma or leukaemia. Not all lumps are lymph nodes; consider the differential diagnosis (Table 8.2). Ask about fever, its duration and pattern. Lymphomas can be a cause of chronic fever, and viral infections such as cytomegalovirus and infectious mononucleosis are associated with haematological abnormalities and fever.


TABLE 8.1 Haematological history

























Major symptoms
Symptoms of anaemia: weakness, tiredness, dyspnoea, fatigue, postural dizziness
Bleeding (menstrual, gastrointestinal, after dental extractions)
Easy bruising, purpura, thrombotic tendency
Lymph gland enlargement
Bone pain
Infection, fever or jaundice
Enlargement of the tongue from amyloidosis
Paraesthesiae (e.g. B12 deficiency)
Skin rash
Weight loss

TABLE 8.2 Differential diagnosis of lymphadenopathy













1 Lipoma—usually large and soft; may not be in lymph node area
2 Abscess—tender and erythematous, may be fluctuant
3 Sebaceous cyst—intradermal location
4 Thyroid nodule—forms part of thyroid gland
5 Secondary to recent immunisation



Treatment


Anaemia may have been treated with iron supplements or B12 injections. Anti-inflammatory drugs or anticoagulants may be the cause of bleeding. Treatment for leukaemia or lymphoma may have involved chemotherapy, radiotherapy, or both; or bone marrow transplant. There may have been blood transfusions in the past.



Past history


A history of gastric surgery or malabsorption may give a clue regarding the underlying cause of an anaemia. Anaemia in patients with systemic disease such as rheumatoid arthritis or uraemia can be multifactorial. Previous blood transfusions may have been required to treat the anaemia. On the other hand, patients with polycythaemia may have had many venesections (page 236).



Social history


A patient’s racial origin is relevant. Thalassaemia is common in people of Mediterranean or southern Asian origin. Rarely, very strict vegetarian diets can result in vitamin B12 deficiency. Find out the patient’s occupation and whether there has been work exposure to toxins such as benzene (risk of leukaemia). Has the patient had previous chemotherapy for a malignancy (drug-related development of leukaemia)? Does the patient drink alcohol?



Family history


There may be a history of thalassaemia or sickle cell anaemia in the family. Haemophilia is a sex-linked recessive disease while von Willebrand’s diseasea is autosomal dominant with incomplete penetrance (Table 8.3).


TABLE 8.3 Causes of ecchymoses











Trauma
Thrombocytopenia or platelet dysfunction (Table 8.4)

Coagulation disorders

Acquired

Vitamin K deficiency (leading to factor II, VII, IX and X deficiency)

Liver disease (impaired synthesis of clotting factors)

Anticoagulants, e.g. heparin, warfarin, proteins with anticoagulant activity

Disseminated intravascular coagulation

Congenital (rarely cause ecchymoses and usually present with haemorrhage)

Haemophilia A (factor VIII deficiency)

Haemophilia B (factor IX deficiency, Christmas disease)

Von Willebrand’s disease (an inherited abnormality of the von Willebrand protein, which is part of the factor VIII complex and causes a defect in platelet adhesion)
Senile ecchymoses (due to loss of skin elasticity)



The haematological examination


Haematological assessment does not depend only on the microscopic examination of the blood constituents. Physical signs, followed by examination of the blood film, can give vital clues about underlying disease. Haematological disease can affect the red blood cells, the white cells, the platelets and other haemostatic mechanisms as well as the mononuclear-phagocyte (reticuloendothelial) system.



Examination anatomy


An important part of the examination involves assessment of all the palpable groups of lymph nodes. As each group is examined its usual drainage area must be kept in mind (Figure 8.1). It follows that whenever an abnormality is discovered anywhere that might be due to infection or malignancy its draining lymph nodes must be examined.


image

Figure 8.1 Usual drainage areas of lymph nodes


Adapted from Epstein O et al, Clinical Examination, 4th edn, Edinburgh: Mosby, 2008.



General appearance


Position the patient as for the gastrointestinal examination—lying on the bed with one pillow. Look for signs of wasting and for pallor (which may be an indication of anaemia—Good signs guide 3.1, page 26). 13 Note the patient’s racial origin (e.g. thalassaemia). If there is any bruising, look at its distribution and extent. Jaundice may be present and can indicate haemolytic anaemia. Scratch marks (following pruritus, which sometimes occurs with lymphoma and myeloproliferative disease) should be noted.



The hands


The detailed examination begins in the usual way with assessment of the hands. Look at the nails for koilonychia—these are dry, brittle, ridged, spoon-shaped nails, which are rarely seen today. They can be due to severe iron deficiency anaemia, although the mechanism is unknown. Occasionally koilonychia may be due to fungal infection. They may also be seen in Raynaud’s phenomenon. Digital infarction (Figure 8.2) may be a sign of abnormal globulins (e.g. cryoglobulinaemia). Pallor of the nail beds may occur in anaemia but is an unreliable sign. Pallor of the palmar creases suggests that the haemoglobin level is less than 70 g/L, but this is also a rather unreliable sign.1


image

Figure 8.2 Digital infarction


Note any changes of rheumatoid or gouty arthritis, or connective tissue disease (Chapter 9). Rheumatoid arthritis, when associated with splenomegaly and neutropenia, is called Felty’s syndromeb: the mechanism of the neutropenia is unknown, but it can result in severe infection. Felty’s syndrome can also be associated with thrombocytopenia (Figure 8.3), haemolytic anaemia, skin pigmentation and leg ulceration. Gouty tophi and arthropathy may be present in the hands. Gout may be a manifestation of a myeloproliferative disease. Connective tissue diseases can cause anaemia because of the associated chronic inflammation.


image

Figure 8.3 Thrombocytopenic purpura


Now take the pulse. A tachycardia may be present. Anaemic patients have an increased cardiac output and compensating tachycardia because of the reduced oxygen-carrying capacity of their blood.


Look for purpura (Figure 8.3), which is really any sort of bruising, due to haemorrhage into the skin. The lesions can vary in size from pinheads called petechiae (from Latin petechia ‘a spot’) (Table 8.4) to large bruises called ecchymoses (Table 8.3).


TABLE 8.4 Causes of petechiae










Thrombocytopenia

Platelet count <100 x 109/L

Increased destruction

Immunological:
    immune thrombocytopenic purpura (ITP)

    systemic lupus erythematosus

    drugs, e.g. quinine, sulfonamides, methyldopa

Non-immunological:
    damage, e.g. prosthetic heart valve

    consumption, e.g. disseminated intravascular coagulation (DIC)

    loss, e.g. haemorrhage

Reduced production

Marrow aplasia, e.g. drugs, chemicals, radiation

Marrow invasion, e.g. carcinoma, myeloma, leukaemia, fibrosis

Sequestration

Hypersplenism

Platelet dysfunction

Congenital or familial

Acquired:
    myeloproliferative disease

    dysproteinaemia

    chronic renal failure, chronic liver disease

    drugs, e.g. aspirin

Bleeding due to small vessel disease

Infection:
    infective endocarditis

    septicaemia (e.g. meningococcal)

    viral exanthemata (e.g. measles)

Drugs, e.g. steroids

Scurvy (vitamin C deficiency)—classically perifollicular purpura on the lower limbs, which is almost diagnostic of this condition

Cushing’s syndrome

Vasculitis:
    polyarteritis nodosa

    Henoch-Schönlein purpura*

Fat embolism

Dysproteinaemia

* Eduard Henoch (1820–1910), professor of paediatrics, Berlin, described this in 1865, and Johannes Schönlein (1793–1864), Berlin physician, described it in 1868.


If the petechiae are raised (palpable purpura), this suggests an underlying systemic vasculitis, where the lesions are painful, or bacteraemia.



The forearms


If thrombocytopenia or capillary fragility is suspected, the Hess testc can be performed.d



Epitrochlear nodes


These must always be palpated. The best method is to flex the patient’s elbow to 90 degrees, abduct the upper arm a little and then place the palm of the right hand under the patient’s right elbow (Figure 8.4). The examiner’s thumb can then be placed over the appropriate area, which is proximal and slightly anterior to the medial epicondyle. This is repeated with the left hand for the other side. An enlarged epitrochlear node is usually pathological. It occurs with local infection, non-Hodgkin’s lymphomae or rarely syphilis. Note the features and different causes as listed in tables 8.5 and 8.6. Certain symptoms and signs suggest that lymphadenopathy may be the result of a significant disease (Good signs guide 8.1).


image

Figure 8.4 Feeling for the epitrochlear lymph node


TABLE 8.5 Characteristics of lymph nodes

















During the palpation of lymph nodes the following features must be considered:
Site
Palpable nodes may be localised to one region (e.g. local infection, early lymphoma) or generalised (e.g. late lymphoma).

The palpable lymph node areas are:
    epitrochlear

    axillary

    cervical and occipital

    supraclavicular

    para-aortic (rarely palpable)

    inguinal

    popliteal

Size

Large nodes are usually abnormal (greater than 1 cm)

Consistency

Hard nodes suggest carcinoma deposits, soft nodes may be normal, and rubbery nodes may be due to lymphoma

Tenderness

This implies infection or acute inflammation

Fixation

Nodes that are fixed to underlying structures are more likely to be infiltrated by carcinoma than mobile nodes

Overlying skin

Inflammation of the overlying skin suggests infection, and tethering to the overlying skin suggests carcinoma

TABLE 8.6 Causes of localised lymphadenopathy













1 Inguinal nodes; infection of lower limb, sexually transmitted disease, abdominal or pelvic malignancy; immunisations
2 Axillary nodes; infections of the upper limb, carcinoma of the breast, disseminated malignancy; immunisations
3 Epitrochlear nodes; infection of the arm, lymphoma, sarcoidosis
4 Left supraclavicular nodes; metastatic malignancy from the chest, abdomen (especially stomach—Troiser’s sign) or pelvis
5 Right supraclavicular nodes; malignancy from the chest or oesophagus

GOOD SIGNS GUIDE 8.1 Factors suggesting lymphadenopathy is associated with significant disease











































































  LR if present LR if absent
Age > 40 2.4 0.4
Weight loss 3.4 0.8
Fever NS NS
Head and neck but not supraclavicular NS NS
Supraclavicular 3.2 0.8
Axillary 0.8 NS
Inguinal 0.6 NS
Size:    
< 4 cm2 0.4
4–9 cm2 NS
> 9 cm2 8.4
Hard texture 3.3 NS
Tender 0.4 1.3
Fixed node 10.9 NS
3 or fewer nodes 0.04
5 or 6 nodes 5.1
7 or more nodes 21.9

From McGee S, Evidence-based physical diagnosis, 2nd edn. St Louis: Saunders, 2007.



Axillary nodes


To palpate these, the examiner raises the patient’s arm and, using the left hand for the right side, pushes his or her fingers as high as possible into the axilla. The patient’s arm is then brought down to rest on the examiner’s forearm. The opposite is done for the other side (Figure 8.5).


image

Figure 8.5 Feeling for the axillary lymph nodes


There are five main groups of axillary nodes: (i) central; (ii) lateral (above and lateral); (iii) pectoral (medial); (iv) infraclavicular; and (v) subscapular (most inferior) (Figure 8.6). An effort should be made to feel for nodes in each of these areas of the axilla.


image

Figure 8.6 The main groups of axillary lymph nodes


A = central; B = lateral; C = pectoral; D = infraclavicular; E = subscapular.



The face


The eyes should be examined for the presence of scleral jaundice, haemorrhage or injection (due to increased prominence of scleral blood vessels, as in polycythaemia). Conjunctival pallor suggests anaemia and is more reliable than examination of the nail beds or palmar creases.3 In northern Europeans the combination of prematurely grey hair and blue eyes may indicate a predisposition to the autoimmune disease pernicious anaemia, where there is a vitamin B12 deficiency due to lack of intrinsic factor secretion by an atrophic gastric mucosa.


The mouth should be examined for hypertrophy of the gums, which may occur with infiltration by leukaemic cells, especially in acute monocytic leukaemia, or with swelling in scurvy. Gum bleeding must also be looked for, and ulceration, infection and haemorrhage of the buccal and pharyngeal mucosa noted. Atrophic glossitis occurs with megaloblastic anaemia or iron deficiency anaemia. Multiple telangiectasiae may appear around the mouth or in the mouth in patients with hereditary haemorrhagic telangiectasia. Look to see if the tonsils are enlarged. Waldeyer’s ringf is a circle of lymphatic tissue in the posterior part of the oropharynx and nasopharynx, and includes the tonsils and adenoids. Sometimes non-Hodgkin’s lymphoma will involve Waldeyer’s tonsillar ring, but Hodgkin’s disease rarely does.



Cervical and supraclavicular nodes


Sit the patient up and examine the cervical nodes from behind. There are eight groups. Attempt to identify each of the groups of nodes with your fingers (Figure 8.7). First palpate the submental node, which lies directly under the chin, and then the submandibular nodes, which are below the angle of the jaw. Next palpate the jugular chain, which lies anterior to the sternomastoid muscle, and then the posterior triangle nodes, which are posterior to the sternomastoid muscle. Palpate the occipital region for occipital nodes and then move to the postauricular node behind the ear and the preauricular node in front of the ear. Finally from the front, with the patient’s shoulders slightly shrugged, feel in the supraclavicular fossa and at the base of the sternocleidomastoid muscle for the supraclavicular nodes. Causes of lymphadenopathy, localised and generalised, are given in Table 8.7. Note that small cervical nodes are often palpable in normal young people.4,5


image

Figure 8.7 Cervical and supraclavicular lymph nodes


1 = submental; 2 = submandibular; 3 = jugular chain; 4 = supraclavicular; 5 = posterior triangle; 6 = postauricular; 7 = preauricular; 8 = occipital.


TABLE 8.7 Causes of lymphadenopathy





Generalised lymphadenopathy
Lymphoma (rubbery and firm)

Leukaemia (e.g. chronic lymphocytic leukaemia, acute lymphocytic leukaemia)

Infections: viral (e.g. infectious mononucleosis, cytomegalovirus, HIV), bacterial (e.g. tuberculosis, brucellosis, syphilis), protozoal (e.g. toxoplasmosis)

Connective tissue diseases: e.g. rheumatoid arthritis, systemic lupus erythematosus

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Mar 25, 2017 | Posted by in PHYSIOLOGY | Comments Off on The haematological system

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