haem Flashcards
Acanthocytes
(Spur/spike cells)
RBCs show many spicules
Liver disease, hyposplenism, abetalipoproteinaemia (rare)
Basophilic RBC stippling
Accelerated erythropoiesis or defective Hb synthesis, small dots at the periphery are seen (rRNA)
Lead poisoning, megaloblastic anaemia, myelodysplasia, liver disease, haemoglobinopathies e.g. thalassaemia
Burr cells
(Echinocyte)
Like a sea urchin with regular spicules
Often an artefact if blood has sat in EDTA prior to film being made.
Uraemia, renal failure, GI bleeding, stomach carcinoma
Heinz bodies
Inclusions on very edge of RBCs due to denatured Hb
Glucose-6-phosphate dehydrogenase deficiency, chronic liver disease
Howell-Jolly bodies
Basophilic (purple spot) nuclear remnants in RBCs
[Note: much bigger purple spots in nucleated RBCs)
Post-splenectomy or hyposplenism (e.g. sickle cell disease)
Megaloblastic anaemia
hereditary spherocytosis
Leucoerythroblastic
A phrase to denote the presence of nucleated red blood cells and myeloid precursors in peripheral blood
Bone marrow infiltration i.e. myelofibrosis, malignancy
Pelger Huet Cells
Hyposegmented neutrophil with 2 lobes like a dumbbell
Pseudo-pelger huet cells are also hypogranular
Congenital (lamin B Receptor mutation)
Acquired (myelogenous leukaemia and myelodysplastic syndromes [pseudo-pelger in MDS])
Polychromasia
Bluish red blood cells due to presence of DNA. Polychromatic cells are usually reticulocytes which are immature RBCs
Usually increased naturally in response to shortened RBC life
↑in haemolytic anaemias
↓aplastic anaemia, chemo
Right shift
Hypermature white cells - hypersegmented polymorphs (>5 lobes to nucleus)
Megaloblastic anaemia, uraemia, liver disease
Rouleaux formation
Red cells stacked on each other
Chronic inflammation,
paraproteinaemia,
multiple myeloma
Schistocytes
Fragmented parts of RBCs – typically irregularly shaped with sharp edges and no central pallor
Microangiopathic anaemia, e.g. DIC, haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura, pre-eclampsia
Spherocytes
Sphere shaped RBCs
Often a little smaller
Hereditary spherocytosis,
Autoimmune Haemolytic Anaemia
Stomatocytes
Central pallor is straight, curved or rod-like shape. RBCs appear as ‘smiling faces’ or ‘fish mouth’
Can be an artefact during slide preparation.
If not: Hereditary stomatocytosis, high alcohol intake, liver disease
Target cells (codocyte)
Bull’s-eye appearance in central pallor
Liver disease, hyposplenism, thalassaemia, IDA
poikilocytosis
(shape) pencil cells
in iron deficiency anaemia
Sideroblastic Anaemia
Ineffective erythropoiesis → iron loading (bone marrow) causing haemosiderosis (endocrine, liver, and cardiac damage due to iron deposition).
* Diagnosis: Ring sideroblasts seen in the bone marrow (erythroid precursors with iron deposited in mitochondria in a ring around the nucleus).
* Causes: myelodysplastic disorders, following chemotherapy, irradiation, alcohol excess, lead excess, anti-TB drugs or myeloproliferative disease.
* Treatment: Remove the cause and consider Pyridoxine (vitamin B6 promotes RBC production). Consider giving EPO.
what does Megaloblastic blood film mean?
= Hypersegmented polymorphs, leukopenia, macrocytosis, anaemia, thrombocytopenia with megaloblasts. Megaloblasts are red cell precursors with an immature nucleus and mature cytoplasm. B12 and folate are required for nucleus maturation.
how to manage pernicious anaemia ?
Treatment: Replenish stores with IM hydroxocobalamin (B12) in 6 injections over 2 weeks.
NICE recommend testing for anti-parietal cell / anti-intrinsic factor antibodies as if there is an autoimmune cause rather than dietary, patients will need 3-monthly IM injections.
inheritance of G6pD
X linked recessive
Pyruvate Kinase Deficiency (inheriatnce, features, treatment)
- Autosomal recessive (but autosomal dominant has been observed with the disorder)
- Clinical features: can be severe neonatal jaundice, splenomegaly, haemolytic anaemia
- Treatment: most do not require treatment (can incl blood transfusion or splenectomy)
how is sickle cell diagnosed ?
Diagnosis: sickle cells and target cells on blood film, sickle solubility test, Hb electrophoresis, Guthrie test (birth) to aid prompt pneumococcal prophylaxis (+FHx)
inheritance of factor 8 and fatcor 9 defiicney
X linkned recessive
diagnosis of haemophilia A?
Diagnosis: ↑APTT, normal PT and ↓ factor VIII assay.
DVT prophylaxis:
*
Daily subcutaneous LMWH (prophylactic dose), TED stockings
o Note: Some DOACs are now licensed for DVT prophylaxis e.g. in post-op ortho patients
Treatment of DVT/PE:
LMWH (treatment dose) followed by Warfarin or Apixaban/Rivaroxaban/Edoxaban (DOACs)
- LMWH stopped once INR in therapeutic range (2-3) (with some DOACs LMWH can be stopped immediately)
- Reason for continuing LMWH while warfarin started: Warfarin also affects protein C/S and often leads to procoagulant state in the first few days before anticoagulant effect
Duration of treatment:
* 3 months minimum
* For clearly provoked VTE consider stop at this point
* Unprovoked VTE needs anticoagulation for 6 months
* Otherwise, needs a clinical decision to be made: there are risk stratification tools used for this. Young men and patients with high baseline D-Dimer are at greater risk.
* Recurrent VTE usually needs lifelong treatment
Warfarin mechnaism
*
Inhibits the vitamin K epoxide reductase enzyme responsible for regenerating the active form of vitamin K and therefore inhibits the synthesis of factors 2, 7, 9, 10 and proteins C, S and Z
Heparin mechanism
- Potentiates antithrombin III which inactivates thrombin, and factors 9, 10, 11
haem changes in pregnancy
plasma volume increase
red cell mass increase
MCV increase
WCC increase
haemoglobin decrease
haematocrit decrease
platelets decrease
Auer rods
acute myeloid leukaemia
Auer rods are cytoplasmic inclusions containing enzymes such as MPO
t(9;22) BCR-ABL1 (Philadelphia chromosome)
chronic myleoid leukaemia
massive splenomegaly in blood cancer what is it?
chronic myeloid leukaemia
common mutation in CML
- Ph+ve (Philadelphia chromosome) in 80% = chromosomal translocation (9;22)
o Using FISH - PCR for BCR-ABL (Philadelphia Chr) fusion gene
o Monitor disease and therapeutic response by monitoring BCR-ABL levels
chemo for chronic myeloid leukaemia
Tx = imatinib (BCR-ABL tyrosine kinase inhibitor
Richter’s transformation
– whereby CLL transforms to more aggressive large cell lymphoma
smear cells
CLL
staging for CLL
Binet Staging A, B & C (Rai Staging I-IV could also be used)
Stage A
o High WBC
o <3 groups of enlarged lymph nodes
o Usually no treatment required
Stage B
o >3 groups of enlarged lymph nodes
Stage C
o Anaemia or thrombocytopenia
different types of non-hodgkins lymphoma and how aggressive / bad they are ?
o High Grade
Very Aggressive – Burkitt’s
Aggressive – Diffuse Large B-Cell, Mantle Cell
o Low Grade
Indolent – Follicular, Marginal Zone, Small Lymphocytic
- HIGHER GRADE LYMPHOMAS ARE EASIER TO TREAT!
staging of non hodskins lymphoma
- Staging Lugano
“Starry sky” appearance on blood film
burkitt’s lymphoma (non hodkins lymphoma)
NOTE: starry sky describes macrophages filled with cellular debris
“Angular/
clefted nuclei”
Mantle cell lymphoma
“Sheets of large lymphoid cells”
Diffuse Large
B-cell (DLBC)
“Follicular pattern”
“Nodular appearance”
Follicular
HTLV-1 infection
Adult T cell leukaemia/lymphoma
lymphoma Associated with mycosis fungoides
Cutaneous T Cell Lymphoma
t(8;14)
burkitts
t(11;14)
mantle cell
t(14;18)
follicular
t(2;5)
anaplastic large cell lymphoma
what is multiple myeloma - what proteins invovled ?
Multiple Myeloma: neoplasia of plasma cells (effector B cells antibodies) of BM.
Production of monoclonal immunoglobulin - “paraprotein” → IgG most common.
Middle-Aged to Elderly.
Increased incidence in Afro-Caribbeans.
Myelodysplastic Syndromes
- Characterised by: peripheral cytopenia; qualitative abnormalities of cell maturation; risk of AML transformation
- Typically seen in the elderly; symptoms usually develop over weeks/months (incidental)
- By definition all patients have <20% blasts (>20% blasts = acute leukaemia)
Clinical Features
* BM failure and cytopenias infection, bleeding, fatigue
* Hypercellular BM
* Defective cells:
o RBCs e.g. ring sideroblasts (abn nucleated blast surrounded by iron granule ring)
o WBCs – hypogranulation, Pseudo-Pelger-huet anomaly (hyposegmented neutro)
o Platelets – micromegakaryocytes, hypolobated nuclei
N.B. In the exam – use an ‘investigative approach’ to pick out clues that lead to classification
polycythaemia vera mutation
JAK2 (V617F).
raised red cells causes ?
Primary causes:
* Polycythaemia vera
* Familial polycythaemia
Secondary causes ( EPO):
* Disease states (renal Ca), high altitude, chronic hypoxia e.g. COPD
Relative (Pseudo) Polycythaemia
Red cell mass normal but plasma volume is reduced.
* Dehydration, burns, vomiting, diarrhoea, cigarette smoking
masive icnrease in platelets what is it ?
Essential Thrombocythaemia (or Thrombocytosis)
An MPD where megakaryocytes dominate the BM.
50% associated with JAK2.
Also associated with MPL mutation and CALR.
Clinical features
* Incidental finding in 50%
* Venous and arterial thrombosis (stroke & MI), gangrene and haemorrhage
* Erythromelalgia
* Splenomegaly, dizziness, headaches, visual disturbances
Investigations
* Platelet count >600x109
* Blood film – large platelets and megakaryocyte fragments
* Increased BM megakaryocytes (not reactive)
Treatment
* Aspirin
* Anagrelide – reduce formation of platelets from megakaryocytes
* Hydroxycarbamide
TACO vs TRALI?
trali = fever, no heart failure, normal or negative fluid balance, no peripheral oedema
trali what does it stand for ?
transfusion related acute lung injruy
Taco what does it stand for ?
transfusion associated cirulatory overload
