White Blood Cell and Lymphatic Disorders Flashcards
1. Illustrate the haematopoietic system 2. Knowledge of WBCs and their functions 3. Knowledge of signs and symptoms of WBC disorders 4. Knowledge of indices used to analyse WBC disorders 5. Interpret basic haematological data for WBC disorders 6. Elucidate dental aspects of WBC disorders
Name the three types of granulocytes
- Neutrophils
- Eosinophils
- Basophils, mast cells
Describe the morphology of neutrophils
Lobed nucleus
Granulocytes in cytoplasm
Where are neutrophils located
Mostly circulating; few in tissues except in inflammation
What is the function of neutrophils
Phagocytose and digest engulfed material
Describe the morphology of eosinophils
Non-segmented/bilobed nucleus
Large eosinophilic granules
Where are eosinophils located
Few in tissues except in inflammation or allergies
What is the function of eosinophils
Participate in inflammatory reaction and immunity to parasites
Describe the morphology of basophils
Lobed nucleus
Large basophilic granules
Where are basophils located
They are circulating as mast cells in most tissues
What is the function of basophils
Release histamine and inflammatory-causing chemicals
Name the three types of mononuclear phagocytes
- Monocytes
- Macrophages
- Dendritic cells
Describe the morphology of monocytes and macrophages
Single nucleus
Abundant in cytoplasm
Where are monocytes located
In circulation where they differentiate into macrophages or dendritic cells once they reach tissues
What is the function of monocytes
Phagocytose and digest engulfed materials
Where are macrophages located
In all tissues
What is the function of macrophages
Phagocytose and digest materials
Describe the morphology of dendritic cells
They are branched
Where are dendritic cells located
Initially in all tissues, but migrate to the lymph nodes
What is the function of dendritic cells
To gather antigens from tissues and present it to lymphocytes
Describe the morphology of lymphocytes
Single nucleus
Little cytoplasm before differentiation
Where are lymphocytes located
In lymphoid organs and in circulation
What is the function of lymphocytes
Participate in adaptive immune response
What WBC disorder involves increased production of WBCs
Leucocytosis (WBC > 11 bil/L)
Increased production due to reactive leucocytosis or drugs (prednisolone)
What WBC disorder involves decreased production of WBCs
Leucopenia (WBC < 4 bil/L)
Secondary to aplastic anaemia
Cyclic neutropenia (rare autosomal dominant condition)
Chemotherapy; radiation; HIV drugs
What is leukaemia
The malignant proliferation of WBCs due to;
genetics, radiation, chemotherapy, viruses, myelodysplastic syndromes (not enough healthy blood cells causing blood cancer)
What are the classifications used for leukaemia
- Cell of origin : Lymphoid or Myeloid
2. Cell maturity : Immature (acute) or Mature (chronic)
Define ALL
Acute lymphoblastic leukaemia
Define AML
Acute myeloblastic leukaemia
Define CLL
Chronic lymphocytic leukaemia
Define CML
Chronic myeloid leukaemia
What are the common symptoms of leukaemia
- fevers, frequent infections, night sweats, fatigue
- easy bleeding, general CNS symptoms an GI symptoms
- shortness of breath, painful lymph nodes
- blurred vision, poor gait, bone + joint pain
What are the characteristics of Acute Leukaemia
- Rapid accumulation of dysfunctional immature cells in blood and bone marrow
- Reduction in normal functions of RBCs/WBCs/platelets
- Short history of feeling unwell; neutropenic fever, bleeding and increased blasts in blood film
- Rashes, lumps, gingival enlargement, testiscular swelling, inter cranial pressure (meninges)
What is ALL
Most common childhood leukaemia with peak incidence at ages 2-4
- prognosis is worse in adults
- clinically indistinguishable from AML
- myeloperoxidase; auer rod marker
What is AML
Most common adult acute leukaemia
- poor prognosis in adults and children
- gingival swelling in 20-30% of patients
- nuclear stained TdT marker (DNA polymerase found in lymphoblasts/myeloblasts)
What are the characteristics of Chronic Leukaemia
- Progresses slowly so allows more mature cell production from bone marrow
- Often asymptomatic so found accidentally
- Both forms are more common in adults
- Blood film: increased circulating mature lymphocytes/myeloid cells
What is CLL
It is more common in men
Rare in asians
Mostly B-cell neoplasm
Splenomegaly + lymphadenopathy is common in CLL
What is CML
Most patients are over 40
Associated with Philadelphia chromosome in 90% of cases (were part of chromosome 9 translocates to chromosome 22)
What are the clinical features and oral manifestations of leukaemia
- Decreased RBCs - fatigue, pallor, hard to perform tasks
- Decreased platelets - mucosal bleeding, petechiae, easy bleeding and bruising
- Decreased WBCs - increased infection risk, poor wound healing
- Increased immature, abnormal, dysfunctional WBCs infiltrating organs - enlargement of gingival, lymph nodes, spleen and liver
Management of leukaemia
- Adjunct supportive therapy
- if anaemic give blood transfusion
- if bleeding problems give platelet transfusion
- if increased infection risk give granulocyte colony stimulating factor transfusion (stimulate bone marrow to make WBCs) - Specific treatments
- chemotherapy
- radiotherapy
- targeted therapy
- bone marrow (stem cell) transplantation (matching donor and immunosuppressants needed)
What is a lymphoma
A solid tumour (Hodgkin’s/Non-Hodgkin’s = most)
Why do lymphomas occur
Due to malignant proliferation of lymphocytes within extranodal sites
What are the signs of a lymphoma
- painful lymph node
- non-specific symptoms; fever, chills, weight loss, sweats, lack of energy, pruritus
- swollen cervical lymph nodes and occasional mouth swellings
What causes Hodgkin’s lymphoma
B-cell dysfunction therefore accumulation of abnormal B-cells
What are the risk factors for Hodgkin’s lymphoma
- Male
- Bimodal age distribution; early 20s and >70
- Immunocompromised
- Positive family history
- History of infectious mononucleosis
Describe the clinical presentation of Hodgkin’s lymphoma
- progressive painless enlargement of lymph nodes (neck)
- this pressurises neighbouring ducts
- tiredness/weakness/fatigue
- fever, anorexia, weight loss, flu-like symptoms
- jaundice (when spread to liver), shortness of breath (when spread to lungs), bone pain
- pruritis
How is Hodgkin’s lymphoma testes for
Paplation/visually and then lymph node biopsy which show Reed-Sternberg cells (owl-like)
FBC, radiographs, CT, MRI, PET scans
Stage I lymphoma (description and treatment given)
Single lymph node is involved/ single extra lymphatic
Radiotherapy
Stage II lymphoma (description and treatment given)
2 or more lymph nodes on the same side of diaphragm; may have localised extralymphatics
Radiotherapy
Stage III lymphoma (description and treatment given)
Lymph nodes on both sides of diaphragm; may include spleen/ localised
Radiotherapy/ Quadruple therapy (combined chemotherapy)
Stage IV lymphoma (description and treatment given)
Diffuse extra lymphatic disease (liver, bone marrow, lung, skin)
Bone marrow transplant
Dental presentation of lymphomas (rarely affects mouth)
Cannot distinguish Hodgkins/Non-Hodgkin’s lymphoma
- oral infections
- mucositis/ oral ulceration by cytotoxic drug
- hypo salivation
- anaemia, bleeding tendancies
- impaired resperitary function
LA regional blocks cannot be used if bleeding tendencies so give GA in hospital
What is Non-Hodgkin’s lymphoma
- B/T cell associated
- More common than HL and has a poorer prognosis and occurs at any age
- Can be aggressive (high grade) or non-aggressive (low)
- Greater presentation for EN sites (GIT, CNS)
- Usually multi-focal
- Dental aspects; Waldeyer’s ring (tonsils) is more common that in HL
What is Burkitt’s lymphoma
Highly aggressive NHL associated with EBV
- starry sky pattern Burkitt cells with lipid droplets
Endemic Burkitt’s lymphoma
- African children and young adults
- Boys
- Jaw swellings and abdominoviscero involvement (pain and nausea)
Sporadic Burkitt’s lymphoma
- Western world
- Males
- Greater incidence in HIV/AIDS
What are the causes of multiple myeloma
Proliferation of malignant plasma cells; overabundance of monoclonal paraproteins (M-proteins) produced by defective immunoglobulins
Malignant plasma cells release osteoclast-activating factors which cause bone resorption and pain
This had genetic links
What are the risk factors for multiple myeloma
- > 60
- Males
- African
- Immunosuppression
- Radiation exposure
- Strong family history
Outline the pathogenesis of multiple myeloma
Malignant plasma cells overwhelm the bone marrow and so 3 cell types are damaged as it causes…
- Over production of BJ (Bence Jones) protein
- Decrease in immunoglobulins
- Increased osteoclastic activity
This overwhelm bone marrow
What does the over production of BJ proteins in multiple myeloma cause
- Hyperviscocity
- Amyloidosis
- Renal failure
- M proteins show up as spikes in electrophoresis
What does the decrease in immunoglobulins cause in multiple myeloma
Impaired humoral immunity and increased infection
M proteins show up as a spike in electrophoresis
What does the increased osteoclastic activity in multiple myeloma cause
Multiple punched out lesions affecting large bones e.g. skull
Hyper calcaemia
Pathalogical bone fractures
What does the overwhelmed bone marrow in multiple myeloma lead to
- Leucopenia
- Anaemia
- Thrombocytopenia
What are the other clinical features of multiple myeloma
- Blood hyperviscosity - thrombosis risk
- Weakness
- Visual disturbance
- Bleeding/bruising - petechiae/ecchymosis
- Infection risk
- Osteoporosis
- Amyloidosis (tongue) = abnormal protein infiltration
How is multiple myeloma tested for
- FBC, renal function (urea, electrolytes, creatine)
- Bone profile (serum Ca2+ levels)
- Serum protein electrophoresis (M protein spike)
- Urine BJ protein
- Bone marrow aspiration (total bone survey shows punched out lesions)
- Radiography of affected bone (CT/MRIs)
- Total body bone survey
What is the dental relevance of multiple myeloma
- Dental treatment his complicated by anaemia, infections and hemorrhagic tendencies
- Jaw osteolytic lesions can involving post. mandible
- Root resorption, loosening teeth and pathological fractures
- Oral complications of chemo and radiotherapy
- Risk of MRONJ
- Rare complications include
- gingival bleeding
- oral petechiae
- cranial nerve palsies
- herpes simplex and zoster infections
What dental treatment should be done pre-chemo
Removal of unstable teeth
Treat caries, OH, diet tips
Chlorhexidine prophylaxis
What dental treatment should be done during induction
Prevenetative OH
Antivirals (acyclovir) and anti-fungal (nystatin/flucondzole) prophylaxis
What dental treatment should be done during remission
Continued oral health preventative care
What dental treatment should be done long term
Continued oral health preventative care and monitoring of orofacial and dental development
What causes reactive leucocytosis
Disease, infections, inflammation, neoplastic conditions
It is the elevated count of WBCs
Clinical presentation of acute leukaemia
- short history of feeling unwell
- present with neutropenia (susceptible to infections)
- fever, bleeding tendencies, bruising, anaemia
- organ infiltration can occur: skin, gums, testes, meninges
- leucocytosis = circulating blasts and cytopenias
Clinical presentation of chronic leukaemia
- incidental finding
- long term history of non-specific symptoms
- splenomegaly
- lymphadenopathy
- leucocytosis = circulating mature lymphocytes/myeloid cells
Risk factors for NHL
- Increasing age
- Immunosuppression
- EBV, H.pylori infections
- Exposure to benzene, herbicides, pesticides
- Strong family history of NHL
Immunodeficiency related Burkitt’s lymphoma
Increased in HIV/AIDS
Oran transplants
What is the classic tetrad of clinical features for multiple myeloma
- Calcium elevation (hypercalcaemia)
- Renal failure
- Anaemia
- Bone pain (especially spinal, pathological fractures)
Outline treatment options for multiple myeloma
- Chemotherapy + steroids to increase chemo effectiveness
- Immunomodulatory drugs to inhibit growth and survival of myeloma cells
- Bone marrow transplant
How are the symptoms and complications of multiple myeloma treated
Analgesics, blood transfusion, dialysis, bisphosphonates helps with fractures, radiotherapy, surgery
What is the prognosis for multiple myeloma
Variable; median survival is 3 years
