Haematology and immunological disorders Flashcards by Rodwan Halimi

What is aplastic anaemia?

Body stops producing blood cells (red and white)

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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

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What is Plummer-Vinson syndrome?

A premalignant condition of the oropharynx

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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

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What is polycythaemia Vera?

Chronic myeloproliferative disease characterized by predominant proliferation of erythroid cell line

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What causes polycythaemia vera?

Primary bone marrow dysfunction => Haemorrhage, thrombosis, increased RBC mass

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What are the clinical signs of polycythemia vera?

Mucosal erythema

Glossitis

Gingivitis.

Bleeding-
high doses of anti-platelet / anticoagulant drug therapy.

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What happens in chronic neutropenia?

Breakdown of a number of factors which neutrophils have function in.

This leads to increased risk of several infections

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What infections are people with chronic neutropenia prone to?

Pyogenic infections

Bacterial infections

Recurrent gingivitis

Severe periodontitis

Ulceration

Candidal infections

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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.

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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

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What should be done for patients with ulceration due to neutropenia?

Provide periodontal treatment to reduce the symptoms as much as possible.

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What is thrombocytopaenia?

Abnormally low platelets

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What can cause abnormal platelet function?

NSAIDs

Anti-platelet drugs

Clopidogrel

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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)

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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)

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What are the types of coagulation disorders?

Hereditary:

Von Willebrand disease (most common)

Haemophilia A (Factor VIII)

Haemophilia B (Factor IX)

Acquired:

Liver disease

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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