Haematology Flashcards
What age does myeloma usually present?
Average age is 60
Which infection is associated with primary gastric lymphoma?
Helicobacter Pylori Infection
What are the risk factors for leukaemia?
Radiation Benzene Smoking Prior Blood disorders Certain Genetic Disorders such as Down's Syndrome
How does bone destruction present in Multiple Myeloma?
Bone pain - back ache is most common, osteolytic lesions, pathological fractures, spinal cord compression and hypercalcaemia. “Pepperpot skull”
How does thrombocytopenia present?
Bruising, Bleeding - mucosal usually, rash (due to low platelet count)
Which leukaemia is more common in children andwhich is more common in adults?
Acute Lymphoblastic Leukaemia is more common in children. Think ALL my children.
Acute Myeloid Leukaemia is more common in middle aged and elderly.
What happens to the blood cells in myeloma?
Rouleaux: Blood cells stick together with too much protein
Normocytic Anaemia
High ESR
How is pernicious anaemia treated?
Intramuscular hydroxocobalamin (Vitamin B12)
What can occur if a sickle cell crisis is not managed?
Stroke could occur
Acute Chest Syndrome: Hypoxia, Sickles, Lung Infarction Cycle
What is the treatment for chronic myeloid leukaemia?
Imatinib, a tyrosine kinase inhibitor
Which drugs are used for treatment of myeloma?
Dexamethasone for infections
Bisphosphonates slow down progression of disease (inhibit osteoclastic activity)
Disease treated with an alkylator, steroid and novel agent
What are some of the differential diagnosis of neck lump?
Infective: Cat scratch Disease, Glandular Fever, TB, Viral Disease, Underlying Neck structure, embryology remnant
Which features are common in both Acute Myeloid Leukaemia and Acute Lymphoblastic Leukaemia?
Anaemia, Neutropenia & Thrombocytopenia
ANT
How Is Thalassaemia Treated?
Alpha is not compatible with life
Beta requires lifelong transfusions
What is the name of the protein that can be found in the urine of individuals suffering with multiple myeloma and what does it contain?
Bence Jones Protein (can be kappa or lambda) and contains light chains
What is a lymphoma?
A Malignant Growth of White Blood Cells, Predominantly in lymph nodes. Can also be in blood, bone marrow, liver, spleen etc
What is Haemophilia B (Christmas Disease) ?
Coagulation Deficiency of factor IX - X linked recessive
How is Multiple Myeloma diagnosed?
Two out of the three diagnostic features should be present:
- Paraproteinaemia on serum protein immunofixation or Bence Jones protein in the urine
- Radiological evidence (CT, MRI) of lytic bone lesions
- An increase in bone marrow plasma cells on bone marrow aspirate or trephine biopsy.
How Does anaemia present?
Shortness of Breath, Fatigue
In Hodgkin’s Lymphoma which markers may be seen in the blood?
ESR usually raised
Serum lactate dehydrogenase (LDH) is raised
May be slightly anaemic
How do you manage febrile neutropenia?
1) See Patient
2) Put in a cannula
3) Give antibiotics
How do you manage a sickle cell crisis?
1) See the patient
2) Control the pain
3) Put in a cannula and start fluids
What are the three main symptoms of lymphoma?
Lump on neck, Fatigue, Sweats
What can cause Iron deficiency?
Blood Loss
Increased Demands such as growth and pregnancy
Decreased Absorption (e.g. small bowel disease or post gasterectomy)
Poor Intake
How is advanced Hodgkin’s Lymphoma treated?
Cyclical Combination Chemotherapy - 8 cycles of ABVD
What is polycythaemia?
Increase in Hb, PCV (packed cell volume) and RCC (Red cell count)
Which investigations should be performed when suspcious of Multiple Myeloma?
Blood Count (may show anaemia, thrombocytopenia and leucopenia) - ESR will be high
Serum Biochemistry may show evidence of renal failure and hypercalcaemia.
Serum ß-2 microglobulin and albumin are used in prognosis
What is pernicious anaemia?
An autoimmune condition in which there is atrophic gastritis with loss of parietal cells, hence failure of intrinsic factor production - thus Vit B12 malabsorption
Which Lymphoma has better survival rates?
Hodgkins Lymphoma - 80% curable
What are some of the supportive treatments of Leukaemia?
Correction of Anaemia, Thrombocytopenia & Coagulation abnormalities by administration of blood, platelets and blood products
Treatment of Infection with IV Antibiotics
Prevention of the acute tumour lysis syndrome
What do you do when you suspect spinal cord compression?
1) Keep Patient in Bed
2) Steroids: Dexamethasone
3) Urgent MRI
What is hyperviscosity syndrome?
Malignant plasma cells churn out a lot of Ig. This protein increases the thickness of blood.
What are the symptoms of hyperviscosity syndrome?
Headaches, Neurological Symptoms, Blurred Vision, Fatigue, Mucosal Bleeding, Confusion or altered mental state, Shortness of Breath
Which leukaemia is characterized by the presence of the philadelphia chromosome?
Chronic Myeloid Leukaemia
Which chromosome abnormality is associated with leukaemia?
The Philadelphia chromosome - found in 97% of cases with chronic myeloid leukaemia.
What are the four types of leukaemia?
Acute Myeloid Leukaemia, Acute Lymphoblastic Leukaemia, Chronic Myeloid Leukaemia and Chronic Lymphocytic Leukaemia
How are Hodgkins and Non-Hodgkins lymphoma differentiated?
In Hodgkins lymphoma, histology will show Reed Sternberg Cells (binucleate or multinucleate malignant B lymphocytes)
How does end organ damage present in Myeloma?
CRAB Calcium (High) Renal Failure Anaemia Bone Disease
What causes AKI in Multiple Myeloma?
Deposition of Light Chains in tubules, hypercalcaumia, hyperuricaemia and amyloid deposition in the kidneys
What are the systemic ‘B’ symptoms associated with lymphoma?
Fever, Drenching Night Sweats and Weight Loss (more than 10% in 6 months)
What is tumour lysis syndrome?
Chemo kills cancer cells which release intracellular components as they die, kidney can be overwhelmed by this and crystallisation and renal failure can occur.
How is leukaemia diagnosed?
Finding more than 20% blasts on bone marrow examination
What are some of the causes of microcytic anaemia?
Iron deficiency
Anaemia of Chronic Disease
Thalassaemia
How is a diagnosis made in acute leukaemia?
Blood film & bone marrow aspirate:
Blood count shows anaemia and thrombocytopenia - white cell count usually raised
Blood film shows characteristic leukaemic blast cells
Bone marrow aspirate shows increased cellularity, with high % of abnormal lymphoid or myeloid blast cells.
What does Rituximab do?
Monoclonal Antibody treatment. Targets CD-20 Cells expressed on cell surface of B Cells - delivered via radiotherapy.
How is Hodgkins lymphoma staged Ann Arbour Staging?
I - Involvement of single lymph node region
II - Involvement of 2 or more lymph node regions on same side of the diaphragm
III - Involvement of lymph node regions on both sides of diaphragm
IV - Diffuse or disseminated involvement of one or more extralymphatic organs or tissues
What are some of the clinical features of anaemia?
Fatigue Faintness Breathlessness Skin & Mucous membranes pale Tachycardia Systolic Flow Murmur
Name and give a brief outline of the three main types of haematological malignancy.
Myeloma - malignancy of plasma cells of bone marrow
Lymphoma - B and T cell malignancies ( Hodgkin and non Hodgkins )
Leukaemia - malignant neoplasms of the haemopoietic stem cells
Which cells are malignant in Myeloma?
Plasma Cells
What are some of the causes of normocytic anaemia?
Acute Blood Loss Anaemia of Chronic disease Combined Deficiency (Iron & Folate for example) Marrow Infiltration or fibrosis Endocrine Disease Haemolytic anaemias
How does Neutropenic Sepsis Present?
Fever, Rigors, General MAlaise, Low Blood Pressure, sometimes cardiovascular collapse (suspect particularly if chemotherapy in last 3 months & temperature)
What are some of the causes of macrocytic anaemias?
Vitamin B12 deficiency Folate deficiency Normoblastic Alcohol Increased Reticulocytes (Haemorrhage, haemolysis) Liver Disease Hypothyroidism Drug Therapy
How does Spinal Cord Compression present?
Back pain, Neuropathic pain, leg weakness or numbness, saddle anaesthesia, loss of sphincter control, urinary retention, decreased anal tone, residual bladder volume more than 200ml
Which lymphoma is found most often in African children?
Burkitt’s Lymphoma
What are the 3 main clinical features of Multiple Myeloma?
Bone Destruction
Bone Marrow Infiltration with plasma cells
Acute Kidney Injury
Which blood test measures the size of red blood cells?
MCV - Mean Corpuscular Volume
Which infection is thought to play a role in pathogenesis of Hodkins Lymphoma?
Epstein-Barr Virus (EBV)
What is Von Willebrands Disease?
Von willebrands Factor contributes to platelet adhesion to damaged subendothelium and stabilisation of factor VIII in plasma. Deficiency of vWF leads to defetive platelet function and factor VIII deficiency.
How does bone marrow infiltration with plasma cella present in multiple myeloma?
Anaemia, Infections & Bleeding
Which antibodies/immunoglobulins are most associated with multiple myeloma?
IgG and IgA
Which chromosomes are translocated in Philadelphia chromosome?
9 and 22.
What is thalassaemia?
Globin chain disorder resulting in diminished synthesis of one or more globin chains, reduction in haemoglobin
How is myeloma managed? What is the survival rate?
Palliative Care, Chemotherapy and Autologous Stem Cell Transplantation - median survival is 5 years. Incurable disease
How is Thalassaemia Intermedia treated?
Still need blood transfusions but not as often. Also when too much iron is deposited in organs, chelation is required.
Endocrine Supplementation also required
What will be seen in blood of someone with tumour lysis syndrome?
Hyperkalaemia
Hyperphosphataemia
Hypocalcaemia
Hyperuricaemia
How is early stage Hodgkin’s Lymphoma treated?
ABVD - Brief Chemotherapy followed by involved field irradiation Adriamycin Bleomycin Vinblastine Dacarbazine
What are the haematological emergencies?
Febrile Neutropenia (Sepsis) Spinal Cord Compression Tumour Lysis Syndrome Sickle Cell Chest Crisis Hyperviscosity Syndrome Hypercalcaemia
What happens to immunoglobulins in myeloma?
It stars in bone marrow, only one Immunoglobulin gets copied and there is no differentiation. Light chains are produced.
