**Anaemia- Iron Deficiency
MICROcytic HYPOchromic
Insufficient iron to support Hb production causing a microcytic, hypochromic anaemia (Fe is absorbed in the duodenum) → ↓Ferritin (iron stores used up)
Causes: blood loss, poor diet, malabsorption, excess fe requirement e.g pregnancy
Signs: SOBOE, fatigue, spoon nails (koilonychia), atrophic glossitis, angular chelitis (ulcer corner mouth), pale eyes, palpitations, dizzy
IVX: FBC = low ferritin confirms, low MCV and MCH and low HB
Coeliac screen, urine dip all patients for bladder cancer, endoscopy and h.pylori urea breath test
TX: Ferrous sulphate 200mg TDS for 3 months and Fe rich food
blood transfusion if Hb <80
**Acute non-haemolytic reactions during transfusion
- febrile reactions
- bacterial contamination
- fluid overload
- Transfusion related acute lung injury
- Anaphylaxis
- Adverse reactions to blood products
- Early Reactions (1/1000) within 24hours (SEVERE):
- Febrile Reactions (1-2% of patients)
- From HLA Abs, Multiparous women/Pts w/ multiple Hx of transfusions most @ risk
- Fever, chills, pruritis, urticaria ~ 1/1.5 hrs from transfusion
TX: Slow the transfusion & give Paracetamol - Bacterial Contamination
- ↑Temp, Hypotension, Rigors
- More common in Platelets (stored @ higher temp)
TX: Stop transfusion, call haematologist, take blood cultures, start broad spec Abx - Fluid overload TACO (pulmonary oedema)
- SOB, hypoxia, tachy, Basal creps
TX: 02 and diuretics - Transfusion Related Acute Lung Injury (TRALI)
(acute, noncardiogenic pulmonary Oedema )
ARDS - dyspnoea and cough- CPAP
- White out lung CXR
TX: STOP transfusion - Anaphylaxis - ig E
TX Adrenaline, salbutamol and Chlorphenamine
Macrocytic anaemia (megaloblastic = large rbc)
CF?
Caused by what deficient?
Management:
- Fall in Hb (MCV > 96fL) due to problems of RBC production (Folate = jejunum, B12 = ileum)
a) Megaloblastic = poor DNA synthesis =
● B12 (due to CD, Addison’s) or Folate (diet, malabsorp, leukaemia, hepatitis) deficiency
b) Non-Megaloblastic =
● Alcohol, liver disease, hypothyroid (sev), pregn., reticulocytosis, myelodysplasia, cytotoxic drugs (e.g. azathioprine)
CF: SOBOE, Fatigue, glossitis, angular stomatitis, dizzy
IVX: bloods HB, MCV, Ferritin, LFTS, coeliac screen
Management:
–> Folate deficient: Diet, folic acid 5mg 5 months
B12 def: B12 injections 1mg alternative days 2 weeks then 1mg every 3 months for life
(avoid peripheral neuropathy and sub acute degeneration of spinal cord = SCDSC)
X- Pernicious anaemia - lemon tingue due to pallor and jaundice, IF auto-Ab
Haemolytic anaemias
= Premature breakdown of RBCs before their natural lifespan (120 d)
Can be intravascular (leading to jaundice) or extravascular (due to immune complex formation or RBC defect spleen)
ACQUIRED (Coombs +ve)
- drug induced- penicillin or infective e.g malaria
HEREDITARY
- –> Enzyme defects = G6PD, pyruvate kinase deficiency
- –> membrane defect = spherocytosis or eliptocytosis
CF: dark urine, fatigue + palpitations, splenomegaly, fever (malaria), jaundice
FHx of jaundice or anaemia may instantly point to a hereditary cause,
IVX: Bloods, blood film, urine and coombs test, USS spleen
- Hodgkin’s Lymphomas
M>F
Malignant proliferation of lymphocytes → + Reed-Sternberg cells
RF: young, EBV, SLE, obesity
CF: lymphadenopathy, painful non tender rubbery typically cervial + painful with alcohol
SYX: Pruritis
B syx: Three B symptoms – weight loss > 10% (6 months), fever > 38, night sweats (needing to change clothes)
IVX: Tissue biopsy via LN excision, histology Reed cells.
- Bloods + staging CXT, marrow biopsy.
TX: CHEMO = ABVD
Adrimycin, Bleomycin, Vinblastine, Decarbazine
Myeloma
plasme cell cancer
- Plasma cell dyscrasia (PCD) due to malignant proliferation of plasma cells i.e. neoplasm of bone marrow plasma cells
- a) Diffuse bone marrow infiltration → bone destruction (↑osteoclast/ ↓osteoblast → ↑Ca2+) + marrow failure
b) Secretion of Ig or paraprotein (detectable in urine) → organ dysfunction esp. renal disease
RF: Afrocarribean
SYX:
- -> Bone disease = backache, fractures, vertebral collapse, hypercalacemia
- -> Blood = Anaemia, neutropenia, thrombocytopenia
- -> Renal imparitment
IVX: Blood, FBC, Raised ESR, U+E = ↑Urea ↑Creat ↑Ca2+, Bence-Jones protein urine test
Management: Blood, fluids, abx
Compliations: MSSC and amyloidosis
2 -Disseminated intravascular coagulation
Dysregulation of coagulation + fibrinolysis → widespread clotting → all coagulation factors used up → resultant massive haemorrhages
Causes: malignancy, sepsis, trauma, HELLP Hamolysis Elevated Liver enzymes, Low platelets,
CF: Large bruising, bleeding, petechiae, renal infection, ARDS
IVX: Bloods, prolonged PT and APPT (Bleeding time)
Management: FFP replace clotting factors. Activated protein C
2- Sickle Cell
- RF: afrocarribean
HbSS (homozygous) or HbAS (trait)
CF: presents children 3-6 months
Anaemia + pallor, jaundice + gall stones
Lethargy and growth restrictions
VASO-OCCULSIVE CRISIS = pain
(triggered by cold, infection, dehydration)
tx: fluids and paracetemol
2- Haemophilia
A = 8 = COMMON - severe B = 9 = RARE - Mild
Deficiency of clotting factor 8 or factor 9
X LINKED RECESSIVE
CF:
A- massive bleed into joints and muscles
B - minor bleeds + bruising
IVX: APPT, PT, Thrombin,
Clotting factors
Management: Clotting factors replacement + desmopressin
2- Thalassaemia
A = Chr 16 B = Chr 11 - major or minor
- mutation of either α or β globin chains of Hb
CF: Anaemia + Fatigue
Hepato-splenomegaly Osteopenia + Skull bossing (B-thal majority)
IVX: Bloods - ↑Bilirubin ↑Reticulocytes
FBC: High HbF = β Major, U&Es, LFTs,
Film: Microcytic, Hypochromic cells +/- Target or Multinucleated RBCs Hb Electrophoresis
–> Imaging
-Skull X-ray – may show “hair on end” appearance of skull due to increased bone marrow activity = B-thal major
Management:
- 2-4 weekly Transfusions for anaemia to maintain Hb > 90
- Fe chelation therapy – minimise Fe overload (excess Fe causes hypothyroidism, hypoCa2+, hypogonad)
- Ascorbic acid – increases urinary excretion of iron
2- Thrombophilia
pro-clotting
- Often presents with unprovoked DVT or PE
e. g Factor V leiden, Prothrombin gene mutation, protein C+S def, Antithrombin def, antiphospholipid syndrome.
TX: LMWH or warfarin
2- Thrombocytopenia
2- Pancytopenia
2- Neutropenia
T = a low platelet count < 150 e.g. lots of bruising and bleeding P = low red, white and platelets N = low neutrophils (WBC)
2- Bone marrow replacement
Conditions its used for?
Conditions used for:
- Severe aplastic anaemia (bone marrow failure)
- Leukaemia – a type of cancer affecting white blood cells
- Lymphoma
- Myeloma – plasma cells
- Disorders –sickle cell anaemia, thalassaemia, severe combined immunodeficiency (SCID) and Hurler syndrome
2- Acute lymphoblastc leukaemia
age group?
symptoms?
rapid onset what?
- Always Little people (children)
●Most common malignancy affecting children – peak 2-5 yo
●↑No of Lymphoid Blast cells push other cells out of bone marrow acutely → rapid onset of Anaemic, Neutropenic, thrombocytopenic Sx
●Can progress from CML
Rapid onset marrow failure: Anaemia – dyspnoea, pallor, lethargy Thrombocytopenia – bruising, purpura Neutropenia – recurrent infections ● Systemic Sx: weight loss, fever, malaise ● Bone pain + splenomegaly
2- Chronic myeloid leukaemia
●MATURE Elderly pts. 60-70yo
●Typically granulocytes (basophils, eosinophils, neutrophils)
●Philadelphia chromosome (95%) due to translocation T( 9:22)
●POOR PROGNOSIS
●Can cause AML (80%), ALL (20%)
↓Weight, LoA
Splenomegaly (significant
2- Chronic lymphocytic leukaemia
-● Almost always B Lymphocytes (99%)
- ● Often no unique features
↓Weight, LoA
Bleeding + infections Lymphadenopathy
Blood product types
- Packed Red Cells
- Platelets
- Fresh Frozen Plasma
- Cryoprecipitate
- Whole Bloods
▪ Packed Red Cells – for severe anaemia, should ↑Hb by ~1.25/dL
▪ Platelets – only used when count v low <50
▪ Fresh Frozen Plasma – for clotting disorders, Vit K deficit, Liver disease, DIC or prophylactically in patients undergoing surgery w/ bleeding risk. (i.e. clinically significant w/o major haemorrh.)
▪ Cryoprecipitate – used to replace fibrinogen (usually if <1.5g/L)
▪ Whole Bloods – very rarely used, exchange transfusions
Non-Hodgkin Lymphoma (90%)
all lymphomas w/o Reed-Sternberg cells, typically derived from B-cell lines
SYX: systemic syx LESS severe then hodgkins.
IVX: marrow biopsy, staging via CXR
MANAGEMENT: High grade lymphoma
- Burkitts (childhood disease characterised by jaw lymphadenopaty
- Treat: R-chop REGIME
- high risk tumour lysis sydrome for all blood cancers –> AKI
Acute Myeloid leukaemia
Always Mature people (older)
- -> Systemic Sx
- brusing, bleeding, breathless, fatigue
- -> Bone pain + splenomegaly
- -> Marrow failure
Can progress from CML
Hodgkin vs Non Hodgkin Age Prognosis Associated pathology: Staging
B syx
age: Hodgkin= 30s, NH = 70s
Prognosis: H =higher survivial NH = lower
H : Reed Sternberg NH: MALT and Burkitts
Staging: Arbor for both
B symptoms refer to systemic symptoms of fever, night sweats, and weight loss
Prognosis in Hodgkin’s lymphoma is influenced by?
Age, stage, presence of B symtpoms and elevated ESR.
1st line treatment for patients with chronic myeloid leukaemia?
CML chromasome?
tyrosine kinase inhibitors such as imatinib!!
IMATINIB!
Philadelphia chromosome
