Hematology: Anemia and Leukemia

• Blood disorders are classified into anaemias, leukaemias, other dyscrasias like multiple myeloma and lymphoma, and bleeding disorders such as thrombocytopenia and coagulopathies.

GDC Learning Outcomes

• General and systemic diseases are relevant to oral health.
• Relevant physiology should be described and explained in its application to patient management.
• Abnormalities of the oral cavity and the rest of the patient must be recognised and addressed.
• Patient needs may cause treatment plans to differ, and patients should be referred for appropriate advice.
• The impact of medical and psychological conditions on patients should be explained.
• Local referral networks, clinical guidelines, and policies should be recognised.
• The responsibilities of the dental team as an access point to and from wider healthcare should be recognised.
• All patient care should be underpinned with a preventive approach that contributes to the patient's long-term oral and general health.
• The impact of a patient's periodontal and general health on the overall treatment plan and outcomes should be explained and taken into account.

Aim of Session

• Blood disorders and the implications for dental hygienists/therapists will be described

Learning Outcomes

• Blood disorders classified
• Deficiency anaemias described
• Sickle cell disease and thalassemia described
• Main types of leukaemia listed
• Leukaemia, multiple myeloma, and lymphoma differences described
• The relationship of these conditions to the work of a dental hygienist/therapist explained

Anaemias Overview

• Anaemias are blood disorders

Definition of Anaemia

• A condition where there is reduced oxygen-carrying capacity of the blood
• Hemoglobin is needed to carry oxygen
• This is a common condition
• Normal hemoglobin production involves iron absorption, hemoglobin production using iron and oxygen binding to hemoglobin for delivery to the body.
• Normal hemoglobin values
  • Below 11.5 g/dl for females indicates anemia
  • Below 13.5 g/dl for males indicates anemia

Classifications of Anaemias

• Deficiency Anaemias
  • Iron deficiency
  • B12 deficiency
  • Folate deficiency
• Haemolytic Anaemia
  • Haemoglobinopathies (inherited)
    • Sickle Cell Disease
    • Thalassaemia
  • Acquired haemolytic anaemias, which overlap with anaemia of chronic disease
• Aplastic Anaemia
• Anaemia of Chronic Disease

Iron Deficiency Anaemia

• Definition: a condition where a lack of iron in the body leads to fewer red blood cells
• Cells become smaller, paler and carry less haemoglobin
• Causes
  • Dietary deficiency
  • Chronic blood loss
  • Malabsorption
  • Pregnancy

Clinical Features of Iron Deficiency Anemia

• Fatigue and lethargy
• Breathlessness
• Palpitations
• Pallor
• Headache
• Brittle nails and koilonychia (spoon-shaped nails)

Dental Clinical Features of Iron Deficiency Anemia

• Pale oral mucosa
• Atrophic glossitis
• Recurrent aphthous ulcers
• Angular cheilitis
• Burning mouth syndrome

Management of Iron Deficiency Anemia

• Check medical history
• Detect underlying cause and refer to GP
• Use iron supplements
• Prescribe preventative dental regime
• Ensure Regular maintenance
• Note that patients are more prone to infection

Vitamin B12 Deficiency

• Needed to synthesise DNA and RNA
• B12 absorption depends on Intrinsic Factor secretion by parietal cells and Terminal ileum which absorbs the B12-intrinsic factor complex
• Causes include Diet (meat and dairy). Vegans at highest risk.
• Impaired absorption: Crohn's, Pernicious Anaemia

Clinical Features of Vitamin B12 Deficiency

• Cells have reduced number of RBCs and they become larger
• Develops slowly
• Usual fatigue, lethargy, breathlessness and pallor
• Dental aspects similar to other deficiency anaemia
  • Glossitis
  • Recurrent aphthous ulcers
  • Angular cheilitis
  • Burning mouth syndrome
  • Red beefy tongue

Management of Vitamin B12 Deficiency

• Correct diagnosis
• Replacement of B12 (cobalamin) with regular intra-muscular injections of hydroxocobalamin at regular intervals
• Preventive dental care and regular recall

Folate Deficiency

• Folate is needed to synthesise DNA and RNA, and to build new cells including RBCs
• Folate can be found in fresh leafy and other vegetables
• Folate is absorbed in the small intestine, but has virtually no stores in the body
• Folate deficiency is mostly due to dietary deficiency
• Clinical Features and Management include dental aspects being similar to other deficiency anaemias like glossitis, recurrent aphthous ulcers, angular cheilitis and red beefy tongue
• Folate deficiency in pregnancy can result in fetal neural tube defects like spina bifida, cleft lip & palate
• Treat with folic acid daily by mouth for several months and improve diet.

Haemolytic Anaemias

• Inherited Haemoglobinopathies
• Sickle cell anaemia
• Thalassaemia
• Acquired
• Viral or bacterial infections
• Medicines e.g. penicillin, antimalarial medicines
• Blood cancers and some tumours
• Autoimmune disorders e.g. lupus, rheumatoid arthritis, ulcerative colitis
• An overactive spleen
• Mechanical heart valves that may damage red blood cells as they leave the heart
• A severe reaction to a blood transfusion

Inherited Haemolytic Anaemias

• Are genetically determined disorders of haemoglobin synthesis
• Includes conditions such as Sickle Cell Disease, Sickle Cell Trait, Thalassaemia, Thalassaemia Trait

Sickle Cell Disease

• Autosomal recessive genetic disease where haemoglobin production leads to intermittent acute crises
• It is most common in people of African descent
• A healthy red blood cell is highly deformable
• A sickled red blood cell is rigid and irregularly shaped
• This irregularly shaped blood can cause episodes of severe pain, damage to vital organs and death

Sickle Cell Trait

• A person inherits the sickle cell gene from one parent and the normal type of that gene from the other parent
• Carriers are generally healthy and rarely have any health problems related to the trait
• When two people with trait have a child, that child may inherit sickle genes and have sickle cell disease

Sickle Cell Crisis

• Low oxygen levels may be bought on by exercise, stress, dehydration, infection, trauma and general anaesthetic
• The misshapen red blood cells clog blood vessels, and result in slowing flow of blood causing anoxia
• This causes sudden severe pain, clotting and potentially death

Clinical Dental Features of Sickle Cell Disease

• Papillary atrophy of tongue
• Neuropathic pain and altered sensation
• Aseptic pulp necrosis
• Osteomyelitis
• Delayed dental eruption
• Mucosal pallor due to anaemia
• Bone pain
• Fungal infections

Sickle Cell Management by Dental Clinician

• Sickle Cell Trait: normal treatment except avoid GA
• Sickle Cell Disease: patients are often treated in specialist centres
  • Preventive care dental regime +++
  • Preventing dental infections is essential
  • Use Antibiotic prophylaxis for invasive dental treatment
  • GA is contra-indicated (conscious sedation is fine with caution)
  • Avoid stress. Need good LA for pain control but caution vasoconstrictor
  • Avoid NSAIDs – paracetamol is OK

Thalassaemia Overview

• Occurs mainly in Mediterranean, Middle/Far Eastern or Asian groups
• Characterised by an abnormal amount of haemoglobin
• It is an inherited disease with genes from both parents
• Results in red cells that are fewer and more fragile
• Leading to early haemolysis causing anaemia

Types of Thalassaemia

• Alpha-thalassaemias
  • Mostly commonly found in Asians
  • There are 4 subtypes of varying degrees of severity
  • The major type is lethal in utero or infancy
• Beta-thalassaemias
  • Mainly Mediterranean & Caribbean
  • There are 2 types: homozygous ẞ-thalassaemia major / heterozygous ẞ-thalassaemia minor

Symptoms of Beta-thalassaemia

• Homozygous (major)
  • Chronic anaemia, marrow hyperplasia, skeletal deformities, splenomegaly, cirrhosis, gallstones & iron overload
  • Iron overload causes cardiac problems, liver & pancreatic dysfunction & dry mouth due to deposits in salivary glands
  • Pallor and tiredness
• Heterozygous (minor)
  • Is much more common and usually asymptomatic apart from mild anaemia

Orofacial Manifestations of Thalassaemia

• 'Hair on end' appearance on lateral skull x-rays
• Prominent maxillae, front bossing (bone marrow expansion)
• Spacing and forward drifting of maxillary incisors
• Painful swelling of parotid salivary glands & xerostomia

Thalassaemia Management

• Severe cases are treated in hospital clinics.
• Care with infection control is vital.
• These patients may have had multiple blood transfusions and may have possibly contracted blood borne diseases.
• Implement Preventive dental care regimes.

Acquired Haemolytic Anaemias

• These Anaemias are acquired, they are not inherited
• Possible causes:
  • Some viral or bacterial infections
  • Medicines like penicillin, antimalarial medicines
  • Blood cancers and some tumours
  • Autoimmune disorders like lupus, rheumatoid arthritis, ulcerative colitis
  • An overactive spleen
  • Mechanical heart valves that may damage red blood cells as they leave the heart
  • A severe reaction to a blood transfusion

Aplastic Anaemia

• Occurs when bone marrow is depressed leading to fewer blood cells (all types)
• Causes: idiopathic (autoimmune), genetic, cytotoxic drugs, radiation, certain chemicals, malignancy (leukaemia), and viral infection.
• May rarely be caused by antibiotics, anticonvulsants and sulphonamides
• May present with severe bruising, fatigue and dyspnoea
• Managed by:
  • Removal of cause
  • Immunosuppressant therapy
  • Consider bone marrow transplant

Anaemia of Chronic Disease

• Characterised by anaemia and evidence of immune system activation
• Caused by decreased red blood cell production +/- and/or increased haemolysis
• Commonly found in the following conditions:
  • chronic infections like HIV
  • autoimmune disorders like rheumatoid arthritis
  • chronic diseases like liver or kidney disease
  • malignancy
  • major trauma
  • major surgery or critical illness
  • older adults

Leukaemias Overview

• Leukaemias are blood disorders

Leukaemias Definition

• Malignant proliferation of white blood cell precursors in bone marrow
• Proliferating immature blast cells crowd out other blood cells formed in bone marrow
• Results in anaemia, thrombocytopenia, leukopenia, and a high risk of infection.
• Risk factors ionising radiation, chemicals, genetic predisposition, but often causes are unknown.

Classification of Leukaemias

• Acute lymphoblastic leukaemia
• Acute myeloid leukaemia
• Chronic myeloid leukaemia
• Chronic lymphocytic leukaemia

Acute Lymphoblastic Leukaemia

• Most commonly found in children, young people, and young adults
• Large numbers of immature white blood cells released
• Drop in red blood cells and platelets
• Treatment occurs with chemotherapy, antibiotics, blood transfusions, and stem cell transplant
• Survival rates after treatment as high as 85% in children, lower in adults

Acute Myeloid Leukaemia

• Aggressive cancer of myeloid cells
• Bone marrow makes abnormal myeloblasts (a type of white blood cell), red blood cells, or platelets
• Affects all ages, but risk of developing increases with age
• Most common acute leukaemia affecting adults
• Quickly fatal if left untreated
• Treatment should include chemotherapy, radiotherapy, bone marrow transplant, and/or stem cell transplant

Chronic Myeloid Leukaemia

• This condition is rare
• Mostly affects adults aged 60+
• There is a slow development of the condition
• Increased and unregulated growth of myeloid cells
• Treatment includes tyrosine kinase inhibitors, and sometimes stem cell transplant
• Outlook is generally good
• A 'blast crisis' can occur

Chronic Lymphocytic Leukaemia

• This is the most common leukaemia
• Too many immature lymphocytes are produced
• Mainly affects people over the age of 60
• May not need treatment for years
• Can be Treated with tyrosine kinase therapy, BCL2 inhibitor therapy, monoclonal antibody therapy, chemotherapy, radiotherapy, and/or immunotherapy

Common Symptoms of Leukemia

• Systemic symptoms such as weight loss, fever, and frequent infections
• Lung symptoms include easy shortness of breath
• Muscular weakness
• Bone and joint pain or tenderness
• Psychological fatigue and loss of appetite
• Swelling Lymph nodes
• Spleen and/or liver Enlargement
• Skin problems such as night sweats, easy bleeding and bruising, and Purplish patches or spots

Leukaemia Clinical Features

• Anaemia (fatigue, pallor etc.)
• Thrombocytopenia (purpura, bleeding tendency)
• Liability to infection
• Lymphadenopathy

Leukaemia Clinical Features

• Cervical lymphadenopathy
• Ulcers
• Mucosal pallor
• Gingival hypertrophy (with acute myeloid leukaemia)
• Spontaneous gingival bleeding
• Petechial haemorrhages of palate, lips
• Infections (viral, candidal, bacterial)
• Defer dental treatment until remission
• Chronic illness - get advice from physician
• Prevention++++

Other Dyscrasias

• Other non cancerous blood conditions

Multiple Myeloma

• Cancer where antibody-producing plasma cells grow in an uncontrolled, invasive manner in bone marrow
• Cancerous plasma cells produce faulty antibodies (paraprotein, which makes it hard for the body to fight infections
• Eventually multiple holes or osteolytic lesions - will form in the bones
• Results in bone pain, recurring infection, kidney damage and fatigue

Lymphoma

• Affects cells in the lymphatic system
• Lymphocytes grow out of control and collect in lymph nodes, spleen, other lymph tissues or in neighboring organs
• Patients commonly present with lymph node enlargement like within the neck, groin, and/or armpit
• Sometimes intraoral lesions
• Two main types:
  • Hodgkin's lymphoma
  • Non-Hodgkin's lymphoma

Dental Implications of Blood Disorders

• Those recieving Radiotherapy or chemotherapy
• Consider those with Multiple myeloma patients who may be on long-term bisphosphonates, this would need to be considered due to risk of osteoradionecrosis
• Infections are common (viral, candidal, bacterial)
• Defer dental treatment when appropriate
• Get advice from physician
• Prioritize Prevention++++

Comparison of Leukaemia, Lymphoma and Multiple Myeloma

• Patients with blood cancers often have symptoms common to all three forms of the disease: weakness and fatigue, bone pain, infections, fevers and weight loss
• Leukaemia - starts in the bone marrow but the cancerous cells are discovered circulating in the blood
• Lymphomas are where the cancerous cells commonly aggregate and form tumors in lymphatic tissues
• Myelomas - tumour of the bone marrow. The increased production of plasma cells in the bone marrow result in damage in the bone marrow and destruction of bone.

Summary of Blood Disorders

• Classified blood dyscrasias
• Outlined deficiency anaemias
• Described haemolytic anaemias including sickle cell disease and thalassemia
• Listed the main types of leukaemia
• Described the difference between leukaemia, multiple myeloma and lymphoma
• Explained how these conditions relate to the work of a dental hygienist/ dental therapist