Clinical Advice

This is available on a 24/7 basis by contacting the on-call Haematology SpR or Consultant Haematologist via the Trust Switchboard 01202 303626

Investigation of Bleeding Disorders

Patients with excessive bruising or bleeding should be investigated initially with a full blood count and coagulation screen, giving any clinical details of family history of bleeding, and patients bleeding after surgery, dental extraction etc.

Following these initial screening tests, further appropriate investigation (e.g. for Von Willebrand’s Disease) will be advised and arranged by a Consultant Haematologist either via referral or A&G on ERS.

Known Patients with Bleeding Disorders

Local patients with known bleeding disorders are registered with, and followed up by, the Haemophilia Centre at University Hospitals Dorset.

These patients have open access for bleeding problems

Dr Luke Attwell, Consultant Haematologist, Royal Bournemouth Hospital is the Director of the Haemophilia Service in Dorset

All patients should carry a red bleeding disorder card which contains information about the specific coagulation factor levels and emergency telephone numbers. (Holiday visitors with bleeding disorders should also carry these cards, which should provide the necessary information)

D-Dimers

Following recent enquiries from GPs concerning D-Dimers, Dr Luke Attwell has reviewed the procedure for telephoning D-Dimer results and has prepared the following information:

D-Dimers are breakdown products of cross-linked fibrin, and a marker for increased fibrinolysis

Cause of raised D-Dimers

• Acute thrombosis
• Cancer
• Diabetic ketoacidosis
• Disseminated intravascular coagulation (DIC)
• Infection
• Pregnancy
• Surgery
• Trauma

 

Normal range

0 – 0.5 µg/mL based on the manufacturer’s recommendations. An age adjusted D-dimer is now used. If aged 51 years or older the upper limit of the normal range is calculated by (age ÷ 100). This is reflected on the individual patient reference range.

 

Telephoning D-Dimer results

GP requests for D-Dimers will only be telephoned to the requestor when they exceed a value of 1 µg/mL

 

D-Dimers in the diagnosis of a VTE

The Negative Predictive Value is 99% if used in conjunction with a negative clinical probability score (such as Wells)

It would be a very rare event to have a patient with a venous thromboembolism (VTE) when the D-Dimer is < 0.5 µg/mL

The importance of performing a Wells score cannot be understated

A patient with a normal D-Dimer and low probability Wells score is highly unlikely to have a VTE

A D-Dimer is not required when a patient has a high probability of a VTE such as a Wells score of > 2 in the context of a possible deep venous thrombosis or > 4 in the context of a possible pulmonary embolus or if a patient is taking anticoagulation therapy

These patients will require a scan to confirm or exclude VTE

D-Dimers can return to normal within 4 weeks of suffering a VTE and after starting anticoagulation therapy

D-Dimer production is known to be suppressed in patients receiving anticoagulation therapy e.g. Warfarin, so their measurement can be unreliable in trying to exclude VTE in this context and may be misleading.

For clinical queries contact Dr Luke Attwell – Consultant Haematologist

 

Thrombophilia Guidelines

Guidance

For most unselected individuals in the general population without a personal history of VTE, the risk-benefit analysis does not favour testing for hereditary thrombophilia. Please see trust guidelines for further advice.

A thrombophilia screen includes the following testing: antithrombin, protein C & S levels, factor V Leiden mutation, prothrombin gene mutation and antiphospholipid testing.

Consider thrombophilia testing for the following indications:

1. Patients presenting with venous thrombosis who are < 40 years old and do not have a precipitating cause and are > 3 months from diagnosis of VTE
2. Patients presenting with an unprovoked venous thrombosis who have an apparent thrombosis-prone family (more than one symptomatic family member) and are > 3 months from diagnosis of VTE
3. Patients with a first-degree relative with confirmed inherited thrombophilia
4. Patients presenting with a venous thrombosis at an unusual site without an obvious cause (e.g. mesenteric thrombus or cerebral venous sinus thrombosis) consider testing for APS, MPN and PNH
5. Antiphospholipid antibodies and lupus anticoagulant testing in patients < 50 years old with a stroke and no identified risk factors for arterial disease or in those with recurrent events despite best medical therapy and no other identified causes
6. Consider MPN and PNH testing for arterial stroke if FBC results are suggestive.
7. Antiphospholipid testing in patients with recurrent pregnancy-related morbidity e.g. recurrent miscarriage.

The genetic mutations screened as part of the thrombophilia investigation are Factor V Leiden G1691A and Prothrombin Gene G20210A

 

Malaria

All patients who have a history of travel to or through (including an airport “stop-over”) an area where malaria is endemic, and who present to a general practitioner unwell, should immediately have a blood film checked for malarial parasites.

Patients in whom malaria is suspected should be referred to the Medical Registrar on duty in the Emergency Department immediately. Please indicate on the request form the malarial area of the world visited

Blood samples should be sent in a Full Blood Count tube (Purple cap)

Further films should be inspected if parasites are not found on initial screening and the patient remains unwell

Immediate and appropriate treatment may be lifesaving in Plasmodium falciparum (malignant tertian) malaria

 

Guidance for patients with polycythaemia

The investigation pathway for patients with increased haematocrit in primary and secondary care is given below as a guide

JAK2 should only be requested via the GP if suggested by Haematology as part of Advice and Guidance as the patient should be referred to the Clinical Haematology team if the result is positive

Hct >0.56 (Male), Hct >0.54 (Female) Causes of secondary (including apparent) polycythaemia include hypoxia, for example in chronic lung disease, smoking, diuretic use, androgens, rarely erythropoietin producing tumours. Please repeat, ensuring that the patient is well-hydrated. If persistent and unexplained, please check MPN screen and serum erythropoietin level and discuss with haematology.

 

Sickle Cell Disease Adult Guidelines

Haematology at UHD links with the Wessex & Thames Valley Haemoglobinopathy Coordinating Centre

Guidelines can be found via this link: NSSG Haematology – Adult Haemoglobinopathies

 

Guideline for the Investigation of a Paraprotein

The presence of a paraprotein is common and increases with age. In many cases no underlying cause for the paraprotein will be found. These cases can be labelled as MGUS (monoclonal gammopathy of uncertain significance). However, the presence of a paraprotein may represent more serious conditions such Multiple Myeloma (MM), Waldenstrom’s macroglobulinaemia (WM), Amyloidosis or other types of lymphoma.

In patients who have features suggestive of one of these conditions, full staging including bone marrow biopsy is indicated. However, in an elderly patient group it is often inappropriate to perform full staging investigations in all patients.

Studies have shown that the likelihood of myeloma is small if the paraprotein level is low. In addition, it is well established that there is no long-term benefit in treating such conditions as MM and WM early before symptoms of the condition or investigative evidence of end organ damage occurs.

The finding of acquired hypogammaglobulinaemia (immuneparesis) without the presence of a paraprotein should also be investigated in the same way as a paraprotein.

1. Initial investigation of all paraproteins (IgG, A or M) should include:

• Clinical history specifically to identify bone pain (usually back), recurrent infection, symptoms of hyperviscosity, symptoms of anaemia, symptoms of hypercalcaemia and peripheral neuropathy.
• Patient examination for evidence of anaemia, lymphadenopathy, hepatosplenomegaly, neuropathy and hyperviscosity (fundi).
• FBC, CRP, renal, liver and bone profiles.
• Serum free light chain ratio, LDH and b2microglobulin.

2. Further management of an IgG or IgA monoclonal paraprotein:

• Patients with a low level paraprotein: IgG < 15g/l and IgA < 10g/l who have no evidence of immuneparesis i.e. other immunoglobin levels are normal.

And

• Have a normal serum free light chain ratio (or a kappa/lambda excess <200)

And

• NO evidence of end organ damage:
• Calcium normal
• Renal function normal
• No anaemia or other cytopenia

And

• NO symptoms suspicious of underlying myeloma (bone pain, recurrent infection etc.)

If ALL the above criteria are met, these patients do not require further investigation but should be followed up with paraprotein measurement every 6 months initially.

If after 2 measurements the paraprotein level is stable blood measurements can be reduced to annually.

Indications for haematological referral for further investigations:

• A paraprotein is found and the patient does not meet the above criteria for monitoring in primary care.
• A low level paraprotein is found but evidence of immuneparesis or suspicious clinical symptoms or anaemia, hypercalcaemia or renal impairment are present.

3. Further management of an IgM monoclonal paraprotein:

Patients with a low level paraprotein: IgM < 10g/l who have no evidence of immuneparesis i.e. other immunoglobin levels are normal.

And

• NO evidence of end organ damage:
• Calcium normal
• Renal function normal
• No anaemia, other cytopenia or lymphocytosis

And

• NO symptoms attributable to the IgM paraprotein or suspicious of underlying lymphoproliferative disease (recurrent infection, lymphadenopathy, hepatosplenomegaly, peripheral neuropathy, cold agglutinin disease, hyperviscosity symptoms).

If ALL the above criteria are met, these patients do not require further investigation but should be followed up with paraprotein measurement every 6 months initially.

If after 2 measurements the paraprotein level is stable blood measurements can be reduced to annually.

Indications for haematological referral for further investigations:

• An increase in paraprotein to above the stated levels or any other change resulting in the patient no longer meeting the above criteria for observation only.
• A low level paraprotein is found but evidence of immuneparesis or suspicious clinical symptoms or cytopenia/lymphocytosis etc. are present.

NOTE: Patients with a significant paraprotein can be referred by a GP via the ‘fast-track’ system if they additionally have any of the following;

• Persistant lymphadenopathy
• Hepatosplenomegaly
• Bone pain with anaemia and raised CRP
• X-rays with lytic lesions
• Three or more of fatigue, recurrent infections, weight loss, bone pain, bruising, breathlessness, itching.

For further information contact Haematology secretaries, 0300 019 4790.