Haematology and immunological disorders Flashcards
What is aplastic anaemia?
Body stops producing blood cells (red and white)
What are the signs of aplastic anaemia?
Oral and facial petechiae
Gingival hyperplasia
Spontaneous gingival bleeding
Oral haemorrhagic bullae
Oral candidiasis
Viral infection
Oral ulceration
What is Plummer-Vinson syndrome?
A premalignant condition of the oropharynx
What are the clinical signs of Plummer Vinson syndrome?
Sideropenic dysphagia
Atrophic glossitis
Angular cheilitis
Hyperkeratotic lesions on oral mucosa
Koilonychia (spoon shaped)
Dysphagia
Pharyngeal-osophageal ulcerations
Oesophageal webs
Oesophageal and pharyngeal SCC
What is polycythaemia Vera?
Chronic myeloproliferative disease characterized by predominant proliferation of erythroid cell line
What causes polycythaemia vera?
Primary bone marrow dysfunction => Haemorrhage, thrombosis, increased RBC mass
What are the clinical signs of polycythemia vera?
Mucosal erythema
Glossitis
Gingivitis.
Bleeding-
high doses of anti-platelet / anticoagulant drug therapy.
What happens in chronic neutropenia?
Breakdown of a number of factors which neutrophils have function in.
This leads to increased risk of several infections
What infections are people with chronic neutropenia prone to?
Pyogenic infections
Bacterial infections
Recurrent gingivitis
Severe periodontitis
Ulceration
Candidal infections
What is cyclic neutropenia?
Recurs every 3 weeks for 3 - 5 days of severe neutropenia.
This leads to repetitive episodes of fever, oral ulcers, and infections.
Which dental issues are associated with neutropenia?
Periodontal disease
Gingivitis
Infections
Neutropenia results in an increase in mucosal NO synthesis and this leads to ulcerations in oral mucosa
What should be done for patients with ulceration due to neutropenia?
Provide periodontal treatment to reduce the symptoms as much as possible.
What is thrombocytopaenia?
Abnormally low platelets
What can cause abnormal platelet function?
NSAIDs
Anti-platelet drugs
Clopidogrel
What causes thrombocytopaenia?
Decreased production:
BM deficiency (Aplasia, CT, RT, infection, toxin, drugs, infiltration)
Inefficient erythropoeiesis
Increased destruction:
Non-immunological (DIC, TTP, HUS, Vasculites, ECMO)
Immunological (Drugs, alloimmune (neonatal purpura, autoimmune (ITP, SLE, LP, HIV, HCV)
What are the types of vascular abnormalities?
Hereditary:
Hereditary haemorrhagic telangiactasia (autosomal dominant)
Ehlers Danlos syndrome
Marfan syndrome
Acquired:
vitC deficiency (Defective collagen synthesis leading to vascular fragility)
What are the types of coagulation disorders?
Hereditary:
Von Willebrand disease (most common)
Haemophilia A (Factor VIII)
Haemophilia B (Factor IX)
Acquired:
Liver disease
What are the clinical manifestations of coagulation disorders?
Facial petechiae
Conjunctival haemorrhage
Oral haematoma
Palatal and tongue purpura
Oral haematoma
Palatal and tongue purpura
Epistaxis
Gingival haemorrhage
Post-extraction haemorrhage
Skin and mucosal telangiectasia
Haemarthrosis of TMJ
How is leukaemia classified?
Clinical behaviour: Chronic or acute
Primary haematopoeitic cell line affected: myeloid or lymphoid
What are the adverse outcomes that can arise due to leukaemias?
Herpes
Candidiasis
Mucosal bleeding
Pallor
Inflamed gingivae
The increased herpes and candidiases as well as the pallor and inflamed gingivae are caused by the undergrowth of other cells due to overgrowth of leukaemic cells.
What are the types of lymphoma? What symptoms are associated with each?
Hodgkin’s lymphoma: Reed-sternberg cells, enlarged lymph nodes
Non-Hodgkin’s lymphoma: Associated with chronic inflammatory disease (sjogren’s), lymphadenopathy or extranodal involvement, Gingival or mucosal tissue swelling or masses, toothaches and intrabony deposits
What is a multiple myeloma?
Plasma cell neoplasm
What are the possible oral manifestations of multiple myeloma?
Osteolytic bone lesions: Increased osteoclast formation, osteoblast inhibition induced by MM cells.
Can manifest soft tissue masses that are extramedullary plasmablastic tumors of the jaws. (Amyloid deposits presenting as nodular masses with/without ulceration.)
Plasma cell tumor growth= pain, paraesthesia, swelling, tooth mobility, radiolucency.
What are the types of immunodeficiency?
Primary and acquired
Acquired is often caused by HIV, immunosuppressive therapy, and cytotoxic drugs
What are the causes of primary immunodeficiency?
B-cell defects: Selective IgA deficiency, hypogammaglobulinaemia
T-cell defects: Mucocutaneous candidiasis, DiGeorge syndrome (congenital thymic aplasia)
Combined immunodeficiency: Severe combined immunodeficiency
Phagocytic defects: Chediak-Higashi syndrome (autosomal recessive due to a mutation of a lysosomal trafficking regulator protein)
What is the relevance of B cell defects in dentistry?
Insufficient circulating antibodies, These are needed to prevent spread of odontogenic infections.
What immune system changes result in chronic mucocutaneous candidiasis?
Absent lymphocyte proliferation to candida antigens
Absent or delayed hypersensitivity skin test response to candida antigens.
Impaired macrophage function
Neutrophil chemotactic defects
What are the oral manifestations of immunodeficiency leading to chronic mucocutaneous candidiasis?
Oral candidiasis
Oral mucosal lesions
What happens in digeorge syndrome?
Thymic aplasia causing issue with T-cells and this results in:
Oral candidiasis
Oral and oesophageal candidiasis
HSV
Periodontitis
RAU
Cleft palate (11%)
Bifid uvula
What happens in severe combined immunodeficiency disease?
Both B and T cells are not very functional. Decreased immune response leading to:
Oral and oesophageal candidiasis
HSV
Periodontitis
RAU
What causes severe combined immunodeficiency syndrome?
X-linked or autosomal recessive condition leading to absence of T and B cell function.
What is chronic granulomatous disease?
A type of phagocytic defect where phagocytes are unable to kill some types of bacteria and fungi.
This leads to periodontal disease and recurrent pyogenic infection.
What is Chediak-Higashi syndrome?
Phagocytic defect where there are deficient platelet dense bodies, neutropenia, and defective neutrophil function with abnormal lysosomal inclusions, impaired chemotaxis, and bactericidal activity, and abnormal NK function
What infections can arise as a result of Chediak-Higashi syndrome?
Beta-haemolytic streptococcus
S.Aureus
Gram -ve species
Candida
Aspergillus
How does HIV affect the immune system?
Reduction in CD4+ T cells
What are the opssible outcomes of HIV?
Parotitis
RAU
HSV
Periodontal disease
Kaposi sarcoma
How does advanced liver disease lead to immunodeficiency?
Causes deficiency in complement production
Increased incidence in bacterial infections
How does multiple myeloma lead to immunodeficiencies?
Decreased Ig production
Combatting extracellular bacterial infections becomes harder as a result
How does nephrotic syndrome lead to immunodeficiency?
Loss of Igs due to glomerular leakage of these immunoglobulins
This leads to increased extracellular bacterial infections.
How do corticosteroids affect the immune system?
Decrease synthesis of interleukins and pro-inflammatory cytokines
Suppress cell-mediated immunity
Decreases complement synthesis
How do the immunomodulators tacrolimus, Methotrexate, and alkylating agents cause immunosuppression?
Tacrolimus reduces activation of T cells because it is a calcineurin inhibitor.
Methotrexate interferes with enzymes required for DNA synthesis
Alkylating agents like cyclophosphamide cause DNA strand breakage.
Which biologics can affect immunocompetence?
TNF-α inhibitor- Infliximab
Monoclonal AB against
CD20 on B-cell surface- Rituximab
Who takes immunosuppression medications?
Transplant patients
Chronic grafts vs host disease
Pemphigous, and pemphigoid
What oral manifestations arise from immune suppression drugs?
Candidiasis
Bacterial infections
Dentoalveolar abscesses
Periodontitis
HSV
VZV
EBV
