Haematology Flashcards
Encapsulated Organisms & Hyposplenism
(Organisms, lifelong treatment, vaccination)
Strep Pneumoniae
Haemophilus Influenzae
N.Meningitidis
Need lifelong penicillin & vaccinations
Require yearly influenzae vaccine
5 yearly pneumococcal C vaccine
Hib vaccine, if not given previously
Men C vaccine, if not given previously
Men ACWY vaccine, if travelling to high risk meningitis area
Heparin Induced Thrombocytopenia (HIT)
(Define, Management)
Use of heparin + positive platelet factor 4-heparin complex antibodies
Self limiting
Waldenstrom Macroglobunemia
(Define, Clinical S&S, Gene mutation, Bone marrow biopsy finding)
Raised IgM paraprotein bands
Generalised muscle weakness
Thickens blood -> clogs up spleen/liver -> hepatosplenomegaly
MYD88 and CXCR4 gene mutation
Bone marrow biopsy: Dutcher bodies
Haemophilia A vs Haemophilia B
(Difference and Similarities)
Haemophilia A (80% cases)
- Factor 8 Deficiency
Haemophilia B (20% cases)
- Factor 9 Deficiency
Similarities
- X-linked recessive
- INR & PT normal
- APTT raised
- Defective intrinsic pathway. Hence APTT raised.
MGUS (Monoclonal gammopathy of undetermined significance)
(Characteristics - 4)
- Premalignant to Multiple Myeloma
- M spikes but <3g/dL
- <10% plasma cells in bone marrow
- No end organ damage
(No CRAB - Hypercalcemia, renal failure, Anemia, Bone lesions)
Sideroblastic Anemia
(Pathophysiology, Causes, Investigations)
- X-linked autosomal recessive
- Propoporphyrin synthesis defect
- Low Hb, MCV, TIBC.
High Ferritin, Tsat, Iron - Causes: Lead poisoning, B6 deficiency, Acquired Myelodysplasia
- Peripheral smear: basophilic stippling, target cells
- Bone marrow: Ringed sideroblasts (inability to incorporate Fe into Hb molecule. Accumulates in mitochondria)
Acute Lymphocytic Leukemia (ALL)
(Lab results, clinical presentation, Complications, Mutations, Treatment)
Raised lymphoblasts, reduced B cells and T cells
Raised WBC, Low Hb, Low Plt, Bone pain
Hepatosplenomegaly, Lymphadenopathy, Meningealeukemia, Thymus enlargement
Causes obstruction of trachea (stridor and dyspnea), oesophagus (dysphagia), SVC (SVC compression syndrome)
9:22t (BCR-ABL Philadelphia Chromosome) - more common in adult
12:21t - More common in children
Blood film: Raised lymphoblasts
Treatment: Tyrosine Kinase (Imatinib)
Acute Myeloid Leukemia (AML)
(Lab results, Mutations, Blood film, Hepatosplenomegaly?, APML treatments)
Raised myeloblasts, low Hb, low Plt, Bone pain
(Unable to make neutrophils, Basophils and Eosinophils)
15:17t - Good prognosis. Associated with APML (Acute Promyelocytic Leukemia). RAR Alpha gene. Increased risk of DIC.
Deletion of chromosome 5 or 7- Poor prognosis.
Blood film - Auer rods and increased myeloblasts
No hepatosplenomegaly
APML
- Increased risk of DIC (Raised PT, APTT, D-Dimer, Low plt, Hb, Fibrinogen)
- Treatment: Vitamin A and Arsenic acid
Chronic Lymphocytic Leukemia (CLL)
(Pathophysiology, Presentation, Consequences of raised autoantibodies, Small Lymphocytic Lymphoma, Evan’s Syndrome, Investigation findings on blood film and immunophenotyping, Treatment)
Mature B Lymphocytes but incompetent -> Incompetent plasma cells -> Reduced antibodies (Hypogammaglobulinemia)
Most common in >70 year olds.
No bone pain. Fever, night sweats, lymphadenopathy, hepatosplenomegaly, increased clotting/viscosity
Raised autoantibodies leading to AIHA (destruction of RBCs), ITP (destruction of platelets)
Small lymphocytic lymphoma: Lymphadenopathy + CLL -> High grade DLBL fro Richter transformation.
Evan’s Syndrome: CLL + AIHA + ITP
Blood film
- Smudge/smear cells (crushed lymphocytes).
- >5000 monoclonal B lymphacytes/mcL
Immunophenotyping (*Main diagnostic method)
- CD5+, CD19 +, CD23 +
Treatment
- FCR (Fludarabine, Cyclophosphamide, Rituximab)
- Prophylactic Co-trimoxazole
Chronic Myeloid Leukemia (CML)
(Pathophysiology, Associations, Explain the 3 phases, Bone marrow biopsy findings)
Crazy Massive Large Spleen!
Overactive Myeloid Stem Cells -> Raised Neutrophils, Basophils, Eosinophils, Platelets, +/- RBC
90% are associated with 9:22t (BCRABL Philadelphia Chromosome) -> Imatinib (Tyrosine Kinase Inhibitor)
Chronic Phase (80-85% of patients): Low blast cells (<10%), raised mature cells
Accelerated Phase: Moderate blast cells (10-20%), Low platelets and RBC, Raised mature cells
Blast Phase: CML -> AML. Blast cells >20%
Bone marrow biopsy: Mixture of all stages of granulocytes development
Hodgkin’s Lymphoma
(Presentation, Phases, Staging, Diagnosis)
Painless lymphadenopathy, fever, night sweats, weight loss
LN biopsy: Characteristic Reed Steinberg cells “owl eyes”
Phase 1: Lymphocytes predominant (Best prognosis)
Phase 2: Nodular sclerosis & Fibrotic bands (Most common type at diagnosis)
Phase 3: Mixed cellularity
Phase 4: Lymphocytes depletion (worst prognosis)
Diagnosis:
- Lymph node biopsy - Reed Steinberg cells “owl eyes”
- CT CAP for staging
- Bone marrow biopsy
Stage 1: 1 lymph node infiltration
Stage 2: >2 lymph node infiltration on the same side (above OR below the diaphragm)
Stage 3: Both sides (above AND below diaphragm)
Stage 4: Extralymph nodes infiltration (into organs)
Non Hodgkin’s Lymphoma
(Examples of T-Cell lymphoma, Examples of B-Cell Lymphoma with their progression rate, Extranodal associations, Treatment)
All other types of Lymphoma besides Hodgkin’ Lymphoma
Can be from B cells (more common) or T cells
T-cells (rare)
- Precursor T-cell neoplasm
- Peripheral T-cell neoplasm
B-cells
- Diffuse Large B Cell Lymphoma (aggressive)
- Follicular Lymphoma (slow-growing)
- Burkitt’s Lymphoma (highly aggressive!)
Extranodal Associations
- MALT (stomach) - associated with H-pylori
- Spinal cord compression (Spinal mets)
- Leukemia (Bone marrow)
Treatment: Rituximab
Thrombotic Thrombocytopenic Purpura (TTP)
(Pathophysiology, Presentation, Difference from HUS, Causes, Diagnosis, Treatment)
Deficiency of ADAMTS13 which breaks down large sticky von Willebrand’s factor
Deficient ADAMTS13 -> Increased vWF -> Thrombus formation -> end organ damage + platelet consumption
Pentad of Thrombocytopenia, Microvascular hemolysis, Neurological signs, Renal impairment, Fever
HUS does NOT have neurological signs, and has more severe renal failure, diarrhea/E.Coli. It also occurs more commonly in children.
Causes
- Recent infection (GI or UTI)
- Clopidogrel
- Heparin
- Tumours
Diagnosis:
- Peripheral blood film - fragmented RBC (schistocytes)
- Anti-ADAMTS13 antibodies
Treatment
- Plasma exchange/plasmaphoresis
- Steroids & Rituximab
Von Willebrand Disease
(Pathophysiology, Inheritance pattern, Presentation, Labs, Treatment)
Reduced vW factor -> Impaired platelet adhesion
Autosomal dominant
Petechial skin rash + elevated APTT + reduced Factor 8 activity
Family history of bleeding
Menorrhagia, Bleeding post dental surgery, easy bruising
Low vWF and Factor 8, Raised APTT
FBC and INR normal
Treatment
- Desmopressin (increase release of vWF)
- TXA if actively bleeding
- Factor 8 transfusion
