Haematology

Published on 24/06/2015 by admin

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Last modified 24/06/2015

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ANAEMIA

Case vignette

An 81-year-old female is admitted to the coronary care unit with crescendo angina. She also complains of early satiety, nocturia and pedal oedema. She reports a 10 kg weight loss over 3 months. She has a background history of rheumatoid arthritis. She is currently managed on a non-steroidal antiinflammatory agent and aspirin. On examination she is hypertensive with a pulse rate of 100 bpm. She has significant conjunctival and palmar crease pallor. The ECG shows evidence of left ventricular hypertrophy and diffuse ST segment depression, together with T wave inversion in the inferior leads. Her blood tests reveal an Hb level of 7 g/dL.

Approach to the patient

An Hb level of < 13 g/dL in men and < 12 g/dL in women is considered anaemic in common practice.

Examination

Look for features of anaemia such as palmar crease pallor, conjunctival pallor, tachypnoea, tachycardia and evidence of high-output cardiac failure. Look for signs that suggest the likely aetiology (see box): characteristic copper-hue pigmentation (the combined effect of melanin deposition, icterus and pallor) of the thalassaemia patient with malar hyperplasia and often stunted growth, pigmented lesions in the mouth (Peutz-Jeghers syndrome), scleral icterus (haemolysis), epigastric tenderness (peptic ulcer disease), abdominal distension or mass lesions, splenomegaly, hepatomegaly, bony tenderness (malignancy), lymphadenopathy (lymphoma), impaired cognition, peripheral neuropathy and dorsal column signs (vitamin B12 deficiency), arthropathy (anaemia of chronic disease or gastrointestinal blood loss secondary to NSAID use), slate-grey pigmentation, peripheral arteriovenous fistula, vascular access device or abdominal Tenckhoff catheter (renal anaemia).

Anaemia in the long case patient is often multifactorial. The diagnostic work-up should include a comprehensive battery of tests as guided by the clinical setting. Iron deficiency secondary to chronic blood loss, commonly from the gastrointestinal tract, is the most common cause of anaemia in this group of patients. Other differential diagnoses encountered commonly include anaemia of chronic disease, vitamin B12 and folate deficiency, myelodysplasia, aplastic anaemia, sideroblastic anaemia, myelosuppression, chronic renal failure, myelofibrosis and chronic alcoholism (due to a combination of bone marrow toxicity of alcohol, iron and folate deficiency and gastrointestinal bleeding).

Investigations

Investigation of anaemia should be guided by the clinical picture. The more common conditions should be excluded first. Relevant investigations in the anaemic patient include:

Blood picture: Patients with anaemia of chronic disease usually have normocytic, normochromic anaemia. Those with iron deficiency, thallasaemia or sideroblastic anaemia have microcytic, hypochromic anaemia. Microcytic, hypochromic anaemia is also seen in myelodysplastic syndrome.

Management

Management depends on severity (level of Hb) and the patient’s cardiopulmonary status. Initial steps include identification and correction of reversible, causative factors and replacement of blood with packed cell transfusions and/or the supplementation of haematinics as indicated.
As a general rule, blood transfusion is indicated if Hb is < 7 g/dL. If Hb remains > 10 g/dL, transfusion should be considered only if there is a compelling clinical indication such as precipitation of coronary ischaemia or heart failure.
Gastrointestinal bleeding warrants endoscopy (colonoscopy or gastroscopy) to identify the exact location of the bleeding and for possible therapy or biopsy of suspicious lesions. If gastroscopy and colonoscopy fail to identify the source of bleeding, capsule endoscopy may be warranted, to look for a small intestinal focus.
Patients with chronic renal failure need iron supplementation and at times may need IV iron infusion. Patients with kidney disease may benefit from therapy with erythropoietin or darbapoietin.
It is recommended that patients with chronic kidney disease be maintained at an Hb level of 11 g/dL or more.
Patients with anaemia of chronic disease improve when the underlying disease is well treated. Haematinic replacement and therapy with erythropoietin or darbapoietin is also indicated in this group.
Management of anaemia due to cytotoxic chemotherapy in patients with non-myeloid malignancies is similar to the management of anaemia of chronic disease.

HAEMATOLOGICAL MALIGNANCIES

Haematological malignancies are commonly encountered in the long case. History taking should enquire into the events surrounding the diagnosis, such as presenting symptoms of fatigue, lethargy, weight loss, fever, night sweats, bone pain, how the diagnosis was made, the various investigations that have been carried out, including bone marrow biopsy and/or excision biopsy of lymph nodes, and the treatment given so far, including blood transfusions, chemotherapy and bone marrow or stem cell transplantation. Ask about the side effects of chemotherapy and any episodes of febrile neutropenia, life-threatening sepsis etc. Ask how the patient is coping with the disease, what knowledge they have about the prognosis of the condition, what expectations they have for the future, and the level of social support available.
Following is a discussion of the salient points relevant to the commonly encountered haematological malignancies. The candidate is expected to have an up-to-date knowledge of each disease and to be able to handle wisely the complicated clinical issues associated with the management of these conditions.

MULTIPLE MYELOMA

Case vignette

A 56-year-old man presents with significant lethargy, severe back pain, loss of sensation in the lower extremities and dizziness. He also complains of bleeding gums and frequent blurring of vision. Physical examination reveals mucosal and conjunctival pallor and bony tenderness of the lower back. There are signs of peripheral neuropathy. His blood tests reveal anaemia, renal impairment and hypercalcaemia.

Approach to the patient

Multiple myeloma is a common malignant condition encountered in the long case setting, because patients with multiple myeloma can have many secondary medical complications. Multiple myeloma is due to disordered proliferation of a clone of plasma cells secreting monoclonal immunoglobulins. The clinical presentation of multiple myeloma is variable.

History

Ask about pain, bony fractures, lethargy, fatigue, dyspnoea, mucosal bleeding, fevers and rigors. Enquire about loss of sensation, limb weakness, visual problems and dizziness.
Some common presentations are intractable bone pain, pathological fracture, symptomatic anaemia, pancytopenia, sepsis, renal failure or acute symptomatic hypercalcaemia. Some may present with the hyperviscosity syndrome, with associated neuropathy and coagulopathy (oral and nasal bleeding, blurred vision, headache and vertigo) or amyloidosis. Hyperviscosity syndrome is more common in Waldenström’s macroglobulinaemia, where the paraprotein is IgM. The most common presenting symptom, however, is bone pain, particularly in the vertebral column. Multiple myeloma should be suspected in any patient over the age of 40 years presenting with bone pain, pathological fracture, osteoporosis, lethargy, anaemia and recurrent infections. Incidental discovery of proteinuria, hypercalcaemia, acute renal failure, high ESR or rouleaux formation should alert the clinician to look for multiple myeloma.

Examination

Look for evidence of anaemia and sepsis. Do not forget to look at the temperature chart. Check the mucosa for bleeding. Perform a thorough neurological examination. Look for bony fractures and assess for bone tenderness.

Investigations

1. Full blood count—looking for anaemia or pancytopenia

2. Blood film—looking for a normocytic normochromic picture and rouleaux formation

4. Serum electrolyte profile

5. Renal function indices—looking for renal failure due to light-chain toxicity, sepsis, amyloidosis and hypercalcaemia

6. Serum calcium level and serum albumin level—looking for hypercalcaemia and hypoalbuminaemia

8. Serum uric acid level—can be elevated

9. Serum and urine protein electrophoresis and immunoelectrophoresis—looking for a paraprotein band

10. Skeletal survey—looking for lytic bone lesions

(Adapted from Grogan T M 2001 Plasma cell neoplasms. In: Jaffe E S, Harris N L, Stein H et al (eds) World Health Organization classification of tumours. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARC Press, Lyon, p 142)
(Adapted from Grogan T M 2001 Plasma cell neoplasms. In: Jaffe E S, Harris N L, Stein H et al (eds) World Health Organization classification of tumours. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARC Press, Lyon, p 142)
(From Greipp P R, San Miguel J, Durie B J et al 2005 International staging system for multiple myeloma. Journal of Clinical Oncology 23(15):3412–3420)
(From Greipp P R, San Miguel J, Durie B J et al 2005 International staging system for multiple myeloma. Journal of Clinical Oncology 23(15):3412–3420)