Haematology Flashcards
Anaemia
low haemoglobin (Hb) concentration, which can be either due to a reduced RBC mass or increased plasma volume It is 130 - 180g/L for men and 120 - 160g/L for women
Microcytic + Normocytic + Macrocytic MCV Anaemia
microcytic (MCV < 83 microns)
normocytic (MCV 80-100 microns)
macrocytic (MCV > 100 microns).
Anaemia Symptoms
– Weakness, fatigue and dyspnea
– Pale conjunctiva
– Headaches + light headedness
Anaemia Diagnosis
– If microcytic –> do iron studies
– If macrocytic –> check blood film to distinguish megaloblastic and normoblastic anaemia.
– If normocytic –> look at the reticulocyte count.
Iron deficiency anaemia
microcytic anaemia which occurs due to increased loss of iron, reduced intake or malabsorption.
Iron deficiency anaemia causes
– Blood loss –> GI bleeding or menstruation
– Poor diet
– Malabsorption –> coeliac disease & IBD
Iron deficiency anaemia symptoms
– Subconjunctival Pallor
– Koilonychia (spoon-shaped nails) & Brittle nails
– Pica (disorder where you eat random stuff e.g. dirt)
-
Iron deficiency anaemia diagnosis
– Blood film shows microcytic, hypochromic RBCs
– Iron studies –> Low ferritin, high Total Binding Capacity
Iron deficiency anaemia Treatment
- iron supplements, (ferrous sulphate)
- treat underlying cause
Glucose-6-phosphate dehydrogenase deficiency
X-linked recessive disorder which may present in babies with jaundice, or haemolysis following exposure to oxidative stressors
Glucose-6-phosphate dehydrogenase deficiency Cause
Sulphur-drugs/ NSAIDs
Deficiency of G6PD enzyme -> Less Glutathione -> no protection of RBCs in Oxidative stress -> makes Heinz bodies into bite cells
Glucose-6-phosphate dehydrogenase deficiency Symptom
- Fatigue
- Palpitations
- SOB
- Pallor
Made worse by fava beans
Glucose-6-phosphate dehydrogenase deficiency Diagnosis
- G6PD enzyme assay
- Blood film shows Heinz bodies & Bite cells & Reticulocytes
Sickle Cell Anaemia
autosomal recessive mutation in the b-chain where a glutamate is replaced with valine.
– This leads to production of HbS rather than HbA
Sickle Cell Anaemia Symptoms
- Sequestration
- Acute chest syndrome - Tachypnoea, Wheeze with hypoxia
- Vaso - occlusive crisis
Sickle Cell Anaemia Diagnosis
Hb electrophoresis
Blood Film - sickle cells
Sickle solubility test
Sickle Cell Anaemia Treatment
- Oxygen
- IV fluids
- Analgesia
- hydroxycarbamide
- Prophylactic antibiotics
Immune Thrombocytopenic Purpura (ITP)
autoimmune production of antibodies directed against platelet antigens → less platelet’s
Immune Thrombocytopenic Purpura (ITP) Symptoms
– Purpura and Gum bleeding
– easy bruising
Immune Thrombocytopenic Purpura (ITP) Diagnosis
– Low platelet count + increased megakaryocytes on bone marrow biopsy
– Platelet autoantibodies
Immune Thrombocytopenic Purpura (ITP) Treatment
– 1st line corticosteroids - Prednisolone
- IV immunoglobin to raise platelet count
- IV Azathioprine (immunosuppression)
Thrombotic Thrombocytopenic purpura (TTP)
condition which occurs due to a deficiency of the enzyme which usually cleaves vWF into monomers
– This leads to large, uncleaved vWF giving abnormal platelet adhesion, schistocytes
Thrombotic Thrombocytopenic purpura (TTP) Symptoms
– Haemolytic Anaemia
– Thrombocytopenia
– AKI
– Fever
Thrombotic Thrombocytopenic purpura (TTP) Treatment
Plasma exchange (removes antibodies) + Steroids
Acute Lymphoblastic Leukaemia (ALL)
neoplastic proliferation of immature lymphoid progenitor cells in the bone marrow, which leads to bone marrow failure and tissue infiltration.
Acute Lymphoblastic Leukaemia (ALL) Symptoms
- Tired
- Fever/Night sweats
- Easy bruising/bleeding
- Petechiae
- Bone pain
Acute Lymphoblastic Leukaemia (ALL) Diagnosis
Bone marrow biopsy shows blast cells
Acute Lymphoblastic Leukaemia (ALL) Treatment
– Chemotherapy
– Bone marrow transplant
Acute Myeloid Leukaemia (AML)
proliferation of immature myeloid cells in the bone marrow.
Associations:
→ Downs Syndrome
→ Radiation
Acute Myeloid Leukaemia (AML) Symptoms
- Fever, SOB
- Easy bruising/ bleeding & gum hypertrophy
- Petechiae
- Weight loss
Acute Myeloid Leukaemia (AML) Diagnosis
– Bone marrow biopsy shows blast cells with Auer rods
Acute Myeloid Leukaemia (AML) Treatment
- Blood transfusion
- Allopurinol
- IV antibiotics
- Chemo
- Bone marrow transplant
Chronic Lymphocytic Leukaemia (CLL) & Complication
neoplastic proliferation of malignant B cells which co-express CD5 and CD20.
accumulation of non-functional B cells
Complication: Richter’s syndrome
Chronic Lymphocytic Leukaemia (CLL) Symptom
- Asymptomatic
- Non- tender lymph node swelling
- Sweating
- Fever & Infection
Chronic Lymphocytic Leukaemia (CLL)
diagnosis
Blood film
– Shows smear/smudge cells
Chronic Lymphocytic Leukaemia (CLL) Treatment
Chemo
Monoclonal Antibodies = Rituximab
Bruton kinase Inhibitors = Ibrutinib
Chronic Myeloid Leukaemia (CML)
neoplastic proliferation of mature myeloid cells, such as basophils.
– Due to 9 ➔ 22 chromosome translocation (Philadelphia Chromosome)
Chronic Myeloid Leukaemia (CML) Symptom
→ As ymptomatic
- Tired
- Weight loss
- Night sweats
- Fever
Chronic Myeloid Leukaemia (CML) Diagnosis
Blood film
- Raised myeloid cells
– Shows many WBC at different stages of differentiation
Chronic Myeloid Leukaemia (CML) Treatment
– 1st line is BCR-ABL tyrosine kinase inhibitor = Imatinib
– Bone marrow transplant
Hodgkin Lymphoma (HL)
malignant proliferation of Reed-Sternberg (RS) cells.
Hodgkin Lymphoma (HL) Symptoms
- Painful lymph node swelling
- Alcohol induced Pain
- SOB, Pain
Hodgkin Lymphoma (HL) Diagnosis
– Blood film with Reed Sternberg cells - diagnostic
– FBC = anaemia, high ESR
Hodgkin Lymphoma (HL) Treatment
Chemotherapy : Adriamycin + Bleomycin + Vinblastine + Dacarbazine
Marrow transplant
Hodgkin Lymphoma (HL) Staging
Staging:
1 = singly lymph node
2 = >1 node on same side diaphragm
3 = nodes on both sides of diaphragm
4 = extra nodal site involvement
Non-Hodgkin’s Lymphoma
group of lymphomas without Reed-Sternberg cells
Associated with viral or Bacterial infection:
→ Epstein Barr Virus with Burkitt’s Lymphoma
→ Helicobacter Pylori
→ Hep.C
Non-Hodgkin’s Lymphoma Symptom
– Superficial lymphadenopathy
- fever, night sweats, weight loss
Non-Hodgkin’s Lymphoma Diagnosis
Blood tests – FBC shows raised WCC, LDH, inflammatory markers
Biopsy of the lymph node
Non-Hodgkin’s Lymphoma Treatment
– Chemotherapy and radiotherapy
– Surgery
Polycythaemia
proliferation of RBCs
So increase in haematocrit, RBC count & Haemoglobin concen.
Polycythaemia Symptoms
- Headaches, blurry vision and dizziness + Hypertension
– Itching after hot bath
– Flushed face
– Gum bleeds
Polycythaemia Diagnosis
FBC shows increased hematocrit, raised cell mass + Mutation in JAK2
- Low Serum Erythropoietin
Polycythaemia Treatment
– 1st line is phlebotomy/ Plasmapheresis
– 2nd line is hydroxycarbamide
Multiple Myeloma
Plasma cell dyscrasia
due to abnormal clonal proliferation of plasma cells in the bone marrow Criteria: → Monoclonal Protein in serum/ urine → Lytic bone lesions → Excess plasma cells in bone marrow
Multiple Myeloma Symptom
C = (Hyper) Calcaemia - due to increased osteoclast - mediated bone resorption
R = Renal impairment
A = Anaemia - due to marrow infiltration by tumour
B = Bone - Osteolytic lesions
H = Hyperviscosity - headaches & thrombosis
A = Amyloidosis
I = Infections - (low lgG)
Multiple Myeloma Diagnosis
– Blood –> FBC shows anaemia, Raised Urea/creatinine, Hypercalcaemia, Rouleaux
– Serum Protein Electrophoresis → Bence Jones Protein in urine
– Bone marrow biopsy –> raised plasma cells
Multiple Myeloma treatment
Haematopoietic Stem Cell transplant
Non- chemo. meds = Thalidomide, Dexamethasone
Chemo. meds = Vincristine
Haemophilia A
Genetic deficiency of Factor VIll
Inherited X- linked recessive
Haemophilia A symptoms
- Deep bruising, Swelling in Joints, Epistaxis
Haemophilia A Diagnosis
- Raised Activated Partial Thromboplastin Time & Low Factor VIll assay
- Normal PT, thrombin time and platelet Count
Haemophilia A Treatment
- Desmopressin → raises Factor VIII levels
- Recombinant Factor VIll
Haemophilia B
X- Linked recessive inherited bleeding disorder caused by mutation in Factor lX
Haemophilia B Management
Recombinant Factor lX
Von Willebrand Disease
Inherited cause of abnormal bleeding (haemophilia).
- Autosomal dominant
- Due to deficiency / absence of glycoprotein - Von Willebrand Factor
Von Willebrand Presentation
- Prolonged Bleeding gums when brushing
- Nose bleeds (epistaxis)
- Menorrhagia
- Heavy bleeding during surgery
Von Willebrand diagnosis
- Bleeding assessment tools
- lab investigations - VWF
- history of abnormal bleeding
Von Willebrand Treatment
- Desmopressin → stimulates release of vWF
- vWF infusion
- Factor VIII → infused with plasma- derived vWF
women with heavy periods can be managed by:
→ Tranexamic acid
→ Combined oral Contraceptive pill
HIV
- RNA retrovirus
- Virus enters & destroys CD4 T- helper cells
- Spread through sexual activity, Pregnancy & blood/ body fluids
- Presents with mouth sores & weight loss
HIV testing
- Antibody testing → Screening for p24 antigen
- PCR testing →test for no. of viral copies → viral load
- CD4 count → lower the count = higher risk of infection
HIV Management
- Antiretroviral Therapy
- Nucleoside Reverse Transcriptase Inhibitor
Tenofovir & Emtricitabine
Microcytic anaemia Causes
→ Iron Deficiency anaemia
→ Sideroblastic anaemia
→ Alpha & Beta Thalassaemia
Macrocytic Anaemia causes
→ B12 / Folate deficiency
Haemolytic Anaemia
Anaemia caused by destruction of red blood cells.
Haemolytic Anaemia Causes
→ Glucose 6 Phosphate Deficiency
→ Sickle cell
Glucose 6 Phosphate Deficiency Management
Blood transfusions
Oxygen therapy
Pernicious Anaemia
Autoimmune condition caused by autoantibodies to Intrinsic Factors (made in parietal cells of stomach) → lack of Intrinsic Factor → B12 deficiency
B12 deficiency Causes
→ Gastric causes - Pernicious anaemia
→ Zollinger - Ellison Syndrome
Pernicious Anaemia Diagnosis
Blood tests:
- Low Hb
- High MCV
- Large RBCs
- Low B12
Pernicious Anaemia Management
- Cobalamin
Aplastic Anaemia + Causes
Autosomal recessive, Pancytopenia with a hypocellular marrow.
Acquired Causes: Drugs (NSAIDs), Infection
Inherited Causes: Fanconi ‘s anaemia
Aplastic Anaemia
Pancytopenia with a hypocellular marrow
Aplastic Anaemia Diagnosis
Anaemia Hb <10 g/dL
Thrombocytopenia Platelets < 50
Neutropenia
Aplastic Anaemia Symptoms
- fatigue & Pallor
- Infection
- Easy bruising / bleeding
Haemophilia B Symptoms & Diagnosis
Symptoms:
- Early life bleeding from soft tissues, joints & muscles.
Diagnosis:
Factor lX assay.
Hodgekin Lymphoma Risk Factors
- Epstein Barr Virus
- HIV
- Immunosuppression
- Smoking
Disseminated Intravascular Coagulation
Symptoms
Diagnosis
Inappropriate activation of Clotting cascade.
Sx = Excess bleeding, fever, Confusion
Ix = Thrombocytopenia, Increased Prothrombin time, Decreased Fibrinogen
DIC Pathophysiology
Tissue damage → Release of Tissue Factor → Clot form. & Consumption of Platelets & Coag. Factors → Tissue plasminogen Activator → Increased fibrinolysis → Increased bleeding
