Blood Disorders

• Blood disorders include anaemias, leukaemias, other dyscrasias like multiple myeloma and lymphoma, and bleeding disorders

Anaemias

• Anaemias involve a reduced oxygen-carrying capacity in the blood.
• Females: below 11.5 g/dl is considered anaemic
• Males: below 13.5 g/dl is considered anaemic
• Haemoglobin is essential for carrying oxygen; insufficient or abnormal red blood cells reduce this capacity.

Deficiency Anaemias

• Deficiency anaemias are a result of lacking essential nutrients
• Iron deficiency is a condition where a lack of iron reduces the number of red blood cells; cells are smaller, paler, and carry less haemoglobin.
• Iron Deficiency Causes: dietary deficiency, chronic blood loss, malabsorption, and pregnancy.
• Vitamin B12 deficiency requires B12 to synthesise DNA & RNA
• Vitamin B12 deficiency depends on intrinsic factor secretion by parietal cells
• Vitamin B12 deficiency requires the terminal ileum which absorbs the B12-intrinsic factor complex
• Vitamin B12 deficiency causes can be diet-related (vegans at risk from lacking meat and dairy), or impaired absorption (Crohn's, Pernicious Anaemia).
• Folate (folic acid) is needed to synthesise DNA & RNA and to build new cells including red blood cells
• Folate is found in fresh leafy & other vegetables
• Folate is absorbed from the small intestine
• The body has virtually no stores of folate
• Folate deficiency is mostly due to dietary deficiency

Clinical Features of Anaemias

• Common symptoms include fatigue, lethargy, breathlessness, palpitations, pallor, headache, and brittle nails (koilonychia).
• Pale oral mucosa occurs
• Atrophic glossitis, recurrent aphthous ulcers, angular cheilitis, and burning mouth syndrome are also features.
• Reduced RBCs and enlarged cells occur in Vitamin B12 deficiency, develops slowly, and causes fatigue, pallor, breathlessness with dental changes simlar to other deficient anaemias

Management of Anaemias

• Management involves checking medical history, detecting the underlying cause (refer to GP), and iron supplements
• The approach should involve preventative dental measures, regular maintenance, and awareness that patients are more prone to infection.
• Treatment for deficiency involves correct diagnosis and replacment of B12 with hydroxocobalamin
• Good preventative dental care and regular recall intervals

Folate Deficiency

• Dental signs mimic other deficiency anaemias which include glossitis, recurrent ulcers, angular cheilitis and red beefy tongue
• A condition which can result in fetal neural tube defects such as spina bifida or cleft lip/palate
• Treating is done with daily oral folic acid and improved diet

Haemolytic Anaemias

• Haemolytic Anaemias can be inherited or acquired
• Inherited haemoglobinopathies include sickle cell disease and thalassaemia.
• Acquired haemolytic anaemias include viral/bacterial infections, medicines (penicillin, antimalarials), blood cancers, autoimmune disorders (lupus, rheumatoid arthritis), overactive spleen, mechanical heart valves, and reactions to blood transfusions.

Inherited Haemolytic Anaemias

• They are genetically determined disorders of haemoglobin synthesis.
• The inherited conditions include sickle cell disease/trait and thalassaemia/trait.

Sickle Cell Disease

• It is an autosomal recessive genetic disease affecting haemoglobin production, associated with intermittent acute crises and most common in people of African descent
• Unlike healthy red blood cells (highly deformable disks), affected cells are rigid and irregularly shaped
• Sickle cell can cause episodes of severe pain, damage to vital organs, and death

Sickle Cell Trait

• It is when a person inherits the sickle cell gene from one parent
• People with sickle cell trait are generally healthy carriers with no related health problems.
• If two people with SC trait have a child, the child may inherit sickle genes and have SC disease

Sickle Cell Crisis

• Low oxygen states may be brought on by exercise, stress, dehydration, infection, trauma, and general anaesthetic.
• Misshapen red blood cells clog blood vessels impairing blood flow and causes severe pain, clotting and death

Dental Clinical Features of Sickle Cell

• Tongue: Papillary atrophy
• Neuropathic pain and altered sensation
• Aseptic pulp necrosis
• Osteomyelitis
• Delayed dental eruption
• Mucosal pallor
• Bone pain
• Fungal infections

Sickle Cell Management by Dental Clinician

• Sickle Cell Trait requires normal treatment except to avoid GA
• Sickle Cell Disease often require special treatment centre
• Sickle Cell Disease requires +++ proventive dental care and infection control
• Antibiotic prophylaxis for invasive dental treatment
• GA is contraindicated, but conscious sedation can be used with caution
• Stress avoidance and good LA pain control
• Avoid vasoconstrictors
• Avoid NSAIDs, but paracetamol is okay

Thalassaemia

• Thalassaemia largely occurs in people with Mediterranean, Middle/Far Eastern or Asian ancestries
• Characterised by abnormal amounts of haemoglobin from two parents: is an inherited disease
• Affected red blood cells are fewer and more fragile which can cause early haemolysis and anaemia.

Types of Thalassaemia

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

Symptoms of ẞ-thalassaemia

• Homozygous (major):
  • Chronic anaemia, marrow hyperplasia, skeletal deformities, splenomegaly, cirrhosis, gallstones & iron overload
  • Iron overload causes cardiac problems and deposits in salivary glands, which leads to; liver & pancreatic dysfunction & dry mouth.
  • Pallor and tiredness
• Heterozygous (minor):
  • Much more common & mostly asymptomatic

Orofacial Manifestations of Thalassaemia

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

Thalassaemia - Management

• Management: Severe cases necessitates treatment in hospital clinics
• Care is infection control is cruicial, as multiple blood transfusions occur, and may expose thalassaemia patients to blood borne diseases
• Required is a preventative dental care regimes

Aplastic Anaemia

• Aplastic Aneamia is when bone marrow is depressed which leads to fewer blood cells of all types
• Aplastic Aneamia causes can be idiopathic (autoimmune), genetic, cytotoxic drugs, radiation, certain chemicals, malignancy (leukaemia), and viral infection, with occassional antibiotic causes
• It may present with severe bruising, fatigue, pallor, palpitations, and dyspnoea
• Management is removal of cause, immunosuppressant therapy, and bone marrow transplant

Anaemia of Chronic Disease

• Key features include anaemia and evidence of immune system activation.
• Decreased red blood cell production and increased haemolysis
• Chronic infections (HIV), autoimmune disorders (rheumatoid arthritis), chronic diseases (liver/kidney), malignancy, major trauma/surgery, and older adults

Leukaemias

• Leukaemias are malignant white blood cell precursor proliferation in bone marrow.
• This results in anaemia, thrombocytopenia, leukopenia, and high infection risk
• Risk factors include: ionising radiation, chemicals, genetic predisposition, many unknown causes.
• The immature blast cells impede other blood cells from forming in bone marrow, as well as crowding normal myeloid cells - resulting in anemia and thrombocytopenia

Classification of Leukaemias

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

Acute Lymphoblastic Leukaemia

• It is most common in children, young people, young adults
• Is charaterised by large numbers of immature white blood cells released
• It results in a drop in red blood cells and platelets
• It can be treated with chemotherapy, antibiotics, blood transfusions, stem cell transplant with survival rates after at 85% in children.

Acute Myeloid Leukaemia

• It is a very aggressive cancer of myeloid cells with abnormal myeloblasts
• It primarily affects adults which increases the risk of getting the condition
• Without treatment it is quickly fatal and can be managed with with chemotherapy, radiotherapy, bone marrow and stem cell transplantation

Chronic Myeloid Leukaemia

• Relatively rare but mostly affects adults
• Increased and unregulated growth of myeloid cells
• Can still have a good outlook however “Blast crisis” can occur
• Treatment options include tyrosine kinase inhibitors and sometimes stem ell transplant

Chronic Lymphocytic Leukaemia

• Most common form of leukaemia
• Immature lymphoctes that are produced
• May never require treatment
• Otherwise treatment include tyrosine kinase therapy, BCL2 inhibitor, as well monoclonal and standard options

Common Symptoms of Leukaemia

Includes systemic signs such as Weight loss, Fever, Frequent infections and Psychological signs such as Fatigue, Loss of appetite. The lungs are Easy shortness of breath, muscular components such as Weakness and Bones or joints components featuring Pain or tenderness

General Clinical Features of Leukaemia

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

Dental Clinical Features of Leukeamia

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

Multiple Myeloma

• A cancer in plasma cells that produces faulty antibodies
• It leads to osteolytic lesions (holes) in the bones and can be caused by bone pain, recurring infection and kidney damage and fatigue.
• The cancerous cells produce faulty antibodies (paratrotein) that cannot fight infections

Lymphoma

• Lymphocytes grow out of control and create an aggregate mass leading to Intraoral lesions
• 2 main type of lymphoma, Hodgkin's and Non-Hodgkin's

Dental Implications for Multiple Myeloma and Lymphoma

• Radiotherapy or chemotherapy
• Multiple myeloma patients on bisphosphonates have high osteoradionecrosis risk to the jaw
• Increased risk of bacterial and viral infections
• Defer dental treatment and obtain physicians’ advice
• Excellent prevention is crucial

Leukaemia vs Lymphoma vs Multiple Myeloma

• All blood cancers share common symptoms: weakness, fatigue, bone pain, infections, fevers, weight loss
• Leukaemia starts in marrow, where cells circulate in blood.
• Lymphoma grows in the lymphatic system and aggregates forming tumours
• Myeloma is a bone marrow tumour that over produces cells and causes destruction

Summary of key takeaways

• Blood dyscrasias classified
• Deficiency anaemias outlined
• Haemolytic anaemias described with sickle cell and thalassaemia
• Leukaemia main tyeps listed
• The difference between leukaemia, multiple myeloma and lymphoma described
• The relationship between all conditions and hygienists or dental therapists understood