Haematology Flashcards
background AML
epidemiology
- most common leukemia in elderly
- median age >60
aetiology
- most are idiopathic
- radiation
- benzenes
- alkylating chemotherapy agents
- topoisomerase II inhibitors
- accumulation of mutations with ageing
genes
- associated with bone marrow failure disorders
- > eg fanconis
- somatic cell chromosome aneuploidy
- > eg down syndrome
- cytogenetic abnormalities
- > unbalanced deletions (more common in treatment related)
- > balanced translocations
- > normal karyotype (more common in de novo than treatment related)
pathophys
- accumulation of blasts
- > unable to differentiate into mature neutrophils, platelets or RBCs
- > bone marrow failure
- little correlation in blast % and cytopenia
- > indicates secretion of inhibitory cytokines
- > rather than crowding out of marrow
hx and exam AML
Hx
- risk factors
- bleeding/clotting
- > easy bruising/bleeding
- > past CVA/MI
- infection
- > fevers
- > systems review for infection
- anaemia
- > SOB
- > palpitations
- > dizziness
- > lethargy
- bone pain
Exam
- lymphocytopenia
- > fever
- > source of infection
- anaemia
- > pallor
- > tachypnea
- > tachycardia
- thrombocytopenia/cytosis
- > splenomegaly
- > petechiae/eccymoses
- invasion (myeloid sarcoma)
- > lymphadenopathy
- > hepatomegaly
- > leukemia cutis
- > gum hypertrophy
CML background
epidemiology
- uncommon in children
- > median age >55
aetiology
- no family hx
- no cancer treatment risk etc
- radiation
- > increased with atomic bomb
- > not with chernobyl
pathophys
- philidelphia chromosome in >90%
- > balanced translocation between long arms of 9 and 22
- > formation of chimeric BCR-ABL1 gene product
- BCR-ABL1
- > codes for constitutively active tyrosine kinase
- > excess proliferation and reduced apoptosis of CML cells
findings
- indolent
- > fatigue
- > weight loss
- splenomegaly
- > early satiety
- > RUQ fullness
- blastic conversion late in coarse
- blood
- > leukocytosis or thrombocytosis
- > anaemia
- marrow
- > high M:E
- > blasts <5%
ALL background
epidemiology
-most common neoplasm in kids
aetiology
- syndromes
- > down syndrome = 20x
- human T cell leukemia virus
- > adults
findings
- blood
- > leukocytosis (but often normal or down)
- > anaemia/neutropenia/thrombocytopenia
- marrow
- > blasts >90% of nucleated cells
CLL background
epidemiology
- older age
- > median age >70
aetiology
- family hx strong
- no environmental exposures known
findings
- indolent
- blood
- > raised WCC with lymphocytosis
- > some lymphadenopathy or splenomegaly
complications
- cancers
- > skin cancers most common
- richters transformation
- > transformation to lymphoma
- > most commonly diffuse large b cell
investigations leukemia
FBC -differential ->20% AML has leuckocytosis ->often anaemia in any leukemia -film ->myeloid blasts plus Auer rods = AML EUCs -hypokalaemia/hyperuricaemia ->tumour lysis syndrome -hypercalcaemia with boney invasion LFTs Coags -TT/fibrin degradation products/D dimer ->DIC in APML -fibrinogen ->decreased
marrow aspirate/biopsy
- > 20% blasts confirms diagnosis
- immunohistochemistry (ALL vs AML)
- > AML = + myeloperoxidase
- > ALL = + TdT
- immunophenotyping (flow cytometry) to subclassify
- > eg CD34/34 % expression
- cytogenetics for prognosis
- > 15:17 translocation = good prognosis
AML prognosis and complications
prognosis
- long term survival is infrequent
- 5 year survival = 25%
complications (CD CLIPT)
- chemo
- > secondary cancers
- > cardiomyopathy
- DIC
- CNS leukemia
- leukostasis
- infection
- pancyopenia
- tumour lysis syndrome
- > hyperuricaemia
- > hyperkalaemia
- > hyperphosphataemia
- > AKI
types of Hodgkins lymphoma
Classic (90%)
- immunophenotype
- > CD 15 +
- > usually CD30 +
- > never CD45+
- nodular sclerosing
- > most common/common in younger patients
- > nodular growth seperated by fibrous bands
- > less common RS cells
- > lacunar variant of RS common (multilobulated nuclei with abundant cytoplasm)
- > mixed inflammatory background
- mixed cellularity
- > elderly patients infected with HIV
- > diffuse or vaguely nodular growth
- > absent of prominent fibrous bands
- > RS more common
- > mixed inflammatory background
- lymphocyte rich
- > usually nodular growth
- > RS cells common
- > inflammatory background is mainly lymphocytes
- lymphocyte depleted
- > least common
- > hypocellular with fibrosis and necrosis
- > lots of RS cells
Nodular lymphocytic predominant (10%)
- immunophenotype
- > CD45 +
- > CD15 -
- > rarely CD30+
- lymphocytic and histiocytic cells
- > polylubulated nuclei
- > nucleus appears exploded as popcorn cell
- nodular growth
- background = b cells, t cells, dendritic cells
hx and exam Hodgkins lymphoma
hx
- onset
- > usually insidious (weeks/months)
- B symptoms (40%)
- > fevers
- > drenching night sweats
- > weight loss >10% over 6 months
- lymphadenopathy (non tender)
- > cervical most common
- > mediastinal (cough, sob, retrosternal pain)
- > axillary
- > step wise progression
- generalised pruiritis
- other
- > pel epstein fevers
- > alcohol induced joint pain
- > hepato/splenomegaly symptoms
- > bone pain
- > skin changes
- PMHx
- > previous malignancy
- > chemo/radiation
- > HIV/immunosuppression
- > EBV
- > fmhx
exam
- lymph nodes
- > all including waldeyers ring
- > size, site, number, firmness, mobility
- hepatosplenomegaly
types of non Hodgkins lymphoma
Aggressive b cell
- diffuse large b cell
- > most common (1/3rd)
- > related to EBV infection, immunosuppression and autoimmune disease
- > large, atypical lymphocytes with prominent nucleoli
- burkitts
- > rare in adults
- > common in children
- > homogenous staining of b cells with pale macrophages gives starry night appearance
Indolent b cell
- follicular lymphoma
- > second most common
- > nodules of small lymphocytes
- gastric MALT
Aggressive T cell
-angioimmunoblastic
Indolent T cell
- Adult T cell
- > associated with human t lymphotropic virus 1
non Hodgkins lymphoma hx and exam
hx
- risk factors
- > family hx
- > past malignancies, chemo, radiation
- > pesticides and hair dyes
- > EBV, HTCLV
- autoimmune diseases
- B symptoms
- > more common in aggressive
- lymphadenopathy
- > non step wise progression
- > waxing and waning in indolent
- extranodal
- > GI most common = distress/bowel obstruction
- > lungs = SOB/cough
- > hepatosplenomegaly= abdodiscomfort
- > CNS = ataxia
- > bone pain
exam
- lymph nodes
- pallor (anaemia
- petichiae/purpura (thrombocytopenia)
- extranodal
- > skin
- > jaundice/hepatosplenomegaly
- > EDCSS = neuro signs
investigations lymphoma
FBC -anaemia -thrombocytopenia EUC -treatment LFTs -treatment -ddxs Prognostic -ESR -albumin -lactate dehydrogenase
excisional lymph node biopsy
- occasionally core, never aspirate
- > diagnosis requires RS/LH cell with appropriate background mileui
- classic = RS or variant (eg lacunar)
- > two nucleoli in seperate nuclear lobes
- > owls eye
- NLP= HS
- > popcorn cell
IMMUNOPHENOTYPING
immunohistochemistry
- classic
- > CD15+ mostly
- > CD30+ almost always
- > CD45-
flow cytometry
- > PDL-1 expressed by some RS
- > ligand for immune checkpoint receptors
STAGING
FDG PET CT
-staging
CONSIDER:
bone marrow biopsy
- Stage 1A and 2A
- > unnecessary as low risk of marrow involvement
- Stage 3 and 4
- > PET CT more sensitive so can be avoided
staging lymphoma
lugano classification
- stage 1
- > one lymph node region
- > single extra lymphatic organ site (1E)
- stage 2
- > involvement of two or more lymph node regions on same side of diaphragm
- > contiguous limited involvement of extralymphatic organ (IIE)
- stage 3
- > involvement of lymph regions above and below diaphragm
- stage 4
- non contiguous involvement of extralymphatic tissue
for treatment
- 1 and 2 = early stage
- 3 and 4 = advanced stage
further notation
- b symptoms
- > A = absent
- > B = present
- bulky disease = X
- E only relevant to early stage
non-hodgkins
- no record of b symptoms
- > no effect on prognosis
- usefulness
- > haematogenous spread more common = less utility
- > no difference in treatment between stage 3 and 4
- > main use is identifying patients with early disease
- > hodgkins spreads by contiguous lymphatic involvement
prognosis hodgkins lymphoma
Early stage (unfavourable/favourable)
- ME3 (German Hodgkins Study Group)
- > unfavourable = 1 of following
- > Mediastinal mass large
- > ESR >30 w B symptoms, >50 no B symptoms
- > 3 or more sites
- favourable with treatment
- > survival at 5 years >97%
Advanced stage (International prognostic score)
- HAM LAW 4
- > Haemoglobin
- > Albumin
- > Male gender
- > Lymphocytosis
- > Age >60
- > WCC
- > stage 4
- freedom from progression =
- > 5 factors = 42%
- > 0 factors = 84%
prognosis non hodgkins
IPS (LEAPS 369)
- for DLBC with rituximab
- > Lactate dehydrogenase
- > Extranodal involvement
- > Age >60
- > Performance (ECOG)
- > Stage 4
- 3 year survival
- > 5 factors = 60%
- > 0 factors = 90%
treatment lymphoma
non hodgkins
- RCHOP for 6 cycles
- > rituximab
- > cyclophosphamide
- > doxorubicin hydrochloride
- > vincristine
- > prednisone
- radiation therapy
hodgkins
- DBVD
- > doxorubicin
- > bleomycin
- > vinblastine
- > dacarbazine
- early = DBVD + radiation
- advanced = just DBVD
- > no benefit from radiation
hodgkins vs non hodgkins lymphoma
epidemiology
- non-hodgkins 10x more common
- hodgkins
- > bimodal
- > 20’s and 80’s
- non hodgkins
- > risk increases with age
risk factors
- hodgkins
- > EBV
- > family hx
- non hodgkins
- > chemo, radiation, pesticides, hair dyes
- > EBV, HTCLV
- > immunosuppression and autoimmune
path
- hodgkins
- > monoclonal expansion of mature b cells
- non hodgkins
- > disease of progenitor or mature B or T cells
clinical presentation
- in general
- > hodgkins = indolent
- > non hodgkins = acute with aggressive or waxing waning with indolent
- > both lymphadenopathy and B symptoms
- differences
- > non hodgkins = non stepwise progression and extranodal involvement more common
- > hodgkins = pel epstein fevers, alcohol induced pain, involvement of mediastinum
diagnosis
- investigations similar
- > bone marrow biopsy in non hodgkins but not usually hodgkins
- both by biopsy
- hodgkins
- > RS or LH with background milleu
- non hodgkins
- > histological typical patterns
- > immunophenotyping for T or B cell origin
- > genotyping for monoclonality and typical mutations
staging
- both lugano classification
- > more useful in hodgkins
treatment
- both chemotherapy
- > but different agents
- both radiation
prognosis
->non hodgkins generally much worse
ddx splenomegaly
CHINA
- congestive
- > CCF
- > cirrhosis
- haematological
- > haemolytic anaemia
- > sickle cell
- infection
- > malaria
- > EBV
- > CMV
- > HIV
- neoplasia
- > CML (CLL)
- > lymphoma
- > myelofibrosis
- autoimmune
- > amyloidosis
- > sarcoid
- > RA (feltys)
Massive = M’s
- cMl
- myelofibrosis
- malaria
Platelet disorders
I’M FluID
- Immune thrombocytopaenia
- Microangiopathic haemolytic anaemias
- > HUS
- > TTP
- > DIC
- Functional defects due to
- > liver disease
- > uraemia
- Inherited diseases
- > Bernard Soulier
- Dysproteinaemias and myeloproliferative disorders
disorders vessel wall
Sick Vessels Can Haemorrhage
- Steroids/cushings
- Vasculitis
- Collagen (Ehlers, marfans, scurvy)
- Henloch Shonlein Purpura
Coagulation in chronic liver disease
Bleeding:
Thrombocytopenia -portal hypertension -decreased thrombopoeitin -DIC Decreased FII,VII,IX,X and fibrinogen -hepatocyte failure -vitamin k DIC Dysfibrinogenaemia Systemic fibrinolysis
Clotting: Altered blood flow Damage to vessel walls DIC Decreased protein C, S and antithrombin -hepatocyte failure -vitamin K
ddx bleeding disorder
Von Willebrand Platelet disorders (I'M FluID) Vessel wall (Sick Vessels Can Haemorrhage) Coagulation disorders -Haemophilia A, B and acquired -Factor XI deficiency Vitamin K -deficiency -enzyme disorder Liver disease
Types of WVD
Type 1: decreased vWF antigen. Activity:Atg normal. All multimeres decreased
Type 2A: decreased vWF antigen, mainly High Molecular Weight, and decreased activity. Activity:Atg decreased
Type 2B: Gain of function, increased binding to GPIb by HMW multimeres. Thrombocytopenia, decreased HMW. Activity: Atg decreased
Type 2M: Decreased antigen, across all multimeres, and decreased binding to GPIb. Activity:Atg decreased.
Type 2N: Decreased binding to FVIII. Increased aPTT. Decreased VIII activity: vWF Atg. vWF activity and antigen normal.
Type 3: undetectable antigen
Investigative algorithm WVD
Initial: vWF Atg, Ristocetin Cofactor, FVIII activity assay
vWF activity and antigen reduced if <30%
Determine ration activity:antigen. Reduced if <0.7
If functional, Type 2 A and B can be separated from Type 2 M by electrophoresis (HWM multimere decreased in A/B)
A and B can be separated by RIPA (agglutination increased in B).
Investigations bleeding disorder
FBC (with platelet count), blood film.
aPTT, PT (INR)
Fibrinogen, TT
(if strong bleeding history):
- PFA 100
- vWF Atg (ELISA), ristocetin Cofactor, FVIII activity assay
LFTs,
EUCs
Iron studies
hx bleeding disorder
Fucking Heavy Bleeding is a MEDICAL emergency
- Family Hx (bleeding or disorder)
- Hospitalisations, transfusions, previous episodes
- Bruising, telangectasias, petechiae
- Menorrhagia
- Estrogen (OCP, HRT, pregnancy)
- Drugs
- Iron deficiency
- Challenges (surgery, trauma, dentist)
- Associated medical conditions
- Lab abnormalities previously
Wells criteria DVT
pre-test probability (VT OILCAMP)
- veins (collateral, non varicose)
- tenderness along venous system
- oedema (pitting) > opposite
- immobility >3 day or surgery < 4 weeks
- leg swollen
- calf swollen >3cm opposite
- alternative diagnosis more likely (minus points)
- malignancy < 6months
- paralysis
0 = low = 3% 1-2= moderate = 17% 3-8 = high = 50 to 75%
Blood film MM
Rouleaux formation Anaemia Leukopenia Thrombocytopenia Monoclonal plasma cell -rare on blood film ->presence indicates plasma cell leukemia -readily detected with flow cytometry
Investigations MM
Urinalysis
-sulfosalicylic acid
FBC -differential -blood film EUC CMP -calcium Lactate dehydrogenase Albumin Beta 2 microglobulin CRP/ESR
SPEP/immunofixation
USEP/immunofixation
sFLC
Bone marrow biopsy and aspirate
- HnE/immunophenotyping/FISH
- percent plasma cells
- morphology
- immunophenotype
Cross sectional imaging
- whole body low dose non con CT
- > most accessible
- whole body MRI
- > most sensitive for bone involvement
- whole body FDG PET/CT
- > most sensitive for extramedullary disease
Skeletal survey
- backup if cross sectional contraindicated
- AP chest
- AP C/T/L spine
- AP humeri and femora
- AP pelvis
- AP and lateral of skull
- findings
- > punched out lytic lesions
- > osteopenia
- > fractures
MM criteria
Clonal bone marrow plasma cells >10%
or
Biopsy proven plasmacytoma
+
1. presence of organ impairment (CRAB) -hypercalcaemia -renal dysfunction (1 boney lesion -anaemia -boney lytic lesions
- evidence of inevitable organ impairment
- >60% plasma cells
- involved:uninvolved FLC ratio 100:1
- MRI with >1 focal lesion
M protein usually >30g/L
ddx for MM
Blood dyscrasia (SWAMPS)
- Smouldering MM
- Waldestroms macroglobulinaemia
- AL amyloidosis
- MGUS
- POEMS
- Solitary plasmacytoma
Immunological complication blood transfusion
A Feared Mistake for Haematologists
- Allergic reaction
- Febrile non haemolytic reaction (anti-leukocyte)
- Modulation with immunosuppression
- Haemolytic anaemia (acute and delayed)
Complication blood transfusion
Haematologists Are Very Concerned about Injecting Blood Products
- Hypothermia
- Acute lung injury (leukocytes vs alloantibodies)
- Volume overload
- Citrate toxicity (alkalosis, calcium)
- Immunologic (A Feared Mistake for Haematologists)
- Bleeding due to dilutional coagulopathy
- Purpura (exposure to platelet antigen)
Atherosclerosis pathophys
Fatty streak and intimal thickening
- earliest change
- fatty streak = accumulation of lipid laden macrophages within intima
- intimal thickening = proliferation of smooth muscle cells and proteoglycan matrix within intima
Pathologic intimal thickening
- extracellular lipid pools = lipid deposits, hyaluron and proteoglycans
- no inflammation
Inflammation and plague progression
- deposition of LDL within intima
- LDL becomes oxidised, which leads to increased expression of adhesion molecules on endothelial cells and chemotaxis
- LDL activates TLRs on macrophages and drive inflammation within intima
Fibroatheroma
- collagen cap overlying necrotic core
- fibrous cap made of collagen synthesised by smooth muscle cells
- necrotic core formed by apoptosis of foam cells, which increases plaque size
- haemorrhage into plaque by leaky vasa vasorum also contributes
- as plaque grows there is positive remodelling of artery to increase lumen diameter and compensate for obstruction
Thin fibrous cap
- thought to develop from fibroatheromas though may arise de novo
- thinning may be due to release of MMP that degrade collagen, or release of interferon gamma by T cells and subsequent smooth muscle cell apoptosis or collagen synthesis inhibition
- more vulnerable to rupture
Rupture or erosion
rupture most common
-large necrotic core, thin fibrous cap containing macrophages and T cell, limited smooth muscle cells
-progressive degradation of cap by MMP, smooth muscle apoptosis and shear forces
erosion
-smaller necrotic core, intact fibrous cap, fewer macrophages and T cells and loss of endothelial lining
-greater accumulation of hyaluron and proteoglycan may drive de-epithelialisation
thrombus formation
-in either case, exposure of blood to procoagulation subepithelial tissue, platelet activation/aggregation and coagulation
Urinalysis MM
MM cast nephropathy
- large waxy, laminated casts
- > precipitated light chains and tam-horsfall
- dipstick normal
- sulfosalacylic acid positive
AL amyloidosis
- positive dipstick
- minimal Bence Protein (UPEP/fixation)
MM vs classic mimics
MGUS
- M protein <30g/L
- clonal bone marrow <10%
- no organ damage symptoms
Smouldering MM
- M protein >30
- clonal bone marrow 10-60%
- no symptoms
Waldenstrom macroglobulinaemia
- lymphoplasmacytic lymphoma in bone marrow
- IgM monoclonal gammopathy in blood
- Absence of CD56 in bone marrow plasma cells
- IgM M protein rare in MM
Solitary plasmacytoma
- normal bone marrow
- absence of CRA
Al amyloidosis
- fewer bone plasma cells
- no lytic bone lesions
- modest Bence Jone proteinuria
- positive dipstick
POEMS 1)peripheral neuropathy 2)monoclonal plasma cell disorder 3) -osteosclerotic lesions or -elevated VEGF or -Castlemans disease ->enlarged lymph nodes single region of body 4) with addition of -endocrinopathy -skin changes -organomegaly
ABI procedure and result interpretation
Continuous doppler over dorsalis pedis or posterior tibial
Ankle cuff inflated
Released -> record systolic with return of pulse
Repeat with other pulse on ipsilateral
Repeat above on contralateral
Measure brachial pulse with same method bilaterally
Divide highest pulse for each lower limb by highest brachial
Results
- 0.9-1.3 = normal
- > 1.3 = calcified vessels
- 0.4-0.9 = arterial obstruction, correlated with claudication
- <0.4 = multilevel disease, non healing ulcer, gangrene
MM bone marrow
Percent plasma cells
- determined by HnE staining
- usually >10%
Morphology
- mature plasma cell
- > oval
- > abundant basophilic cytoplasm
- > eccentrically placed nucleus
- > clock face chromatin, no nucleoli
- immature plasma cell
- > dispersed nuclear chromatin
- > prominent nucleoli
- > high N:C
- intracellular Ig accumulations
- > Morula cells (bluish white grape like)
- > Russel bodies (cherry red, round)
- > Flame cells (vermilion staining glycogen rich IgA)
- > Gaucher cells (overstuffed fibrils)
- > crystalline rods
Immunophenotype
- determined by immunohistochemistry, immunoflurescence, flow cytometry
- presence of kappa or lambda in plasma cells, but not both
- normal ratio K:L = 2:1
- > 4:1 or 1:2 = monoclonality
- CD receptors
- > no CD19
- > CD45 rare
- > most CD56 positive
Cytogenetics
- FISH
- no specific changes
MM blood investigation results
Detection of M protein
- SPEP = 80% sensitivity
- SPEP + immunofixation = 90%
- ” “ + light chain assay or UPEP/urine immuno fixation = 97%
- remainder are non secretory MM
Serum and Urine Protein Electrophoresis (SPEP/UPEP)
- > gamma, beta or alpha 2 mobility
- > narrow spike on densitometer tracing
- > dense discrete band on agarose band
Serum immunofixation
- confirms presence of M protein
- determines type of M protein
- > IgG/IgA/IgM/IgD
- > Kappa or Lambda light chains
- possible results
- > heavy chains + light chains
- > light chains alone (Bence Jones)
- reduction in uninvolved Ig in most cases
- > less frequently, reduction of both
Free light chain assay
- anti sera to constant region of Kappa and Lambda light chains
- > measures light chains not bound to heavy chains in serum
- light chain MM detected by abnormality in ratio of K:L
- > detected in >90% of MM
- absolute FLC level also prognostic
Light chain myeloma
- approx 20% of cases
- normal serum proteins
- readily detected by sFLC, UPEP and urine immunofixation
Non secretory
- approx 60% will have abnormal sFLC ratios
- majority of remainder will have M protein detectable in neoplastic cell cytoplasm
- hypogammaglobinaemia seen in 50% of non secretory
hypo proliferative anaemia causes
Marrow damage:
- infiltration
- fibrosis
Decreased production:
- Aplastic
- Iron deficiency
Systemic:
- Renal disease
- Hypothyroidism
features hypo proliferative anaemia
CBC:
normocytic, normochromic anaemia
Reticulocyte count:
low response
Blood film:
nil
Bone marrow:
M:E 2-3
features of proliferative (bleeding/haemolytic) anaemia
CBC:
normocytic, may be macrocytic with reticulocytosis
normochromic (unless iron deficient)
Reticulocyte count: high
Blood film:
polychromatophilic macrocytes
Bone marrow:
nil.
what are ringed sideroblasts
Defect in haem synthesis. Iron is taken up by mitochondria but not incorporated into haem. Iron encrusted mitochondria surround nucleus, forming ring. Seen in myelodysplasia
features maturation defect anaemia
CBC:
Macrocytosis or microcytosis
Normochromic or hypochromic
Reticulocyte count:
low
Blood film: May show specific anomaly
Marrow: M:E of 1, reflecting ineffective erythropoesis.
Howell Jolly bodies
Dense blue circular inclusions in RBC that represent nuclear remnants and defective spleen function
What are spherocytes
small RBC without central pallor. Seen in hereditary spherocytosis and haemolytic anaemias
What are dacrocytes
teardrop shaped RBC. Seen in haemolytic anaemias, iron deficiency, thalassaemia, myelofibrosis, myelodysplastic syndrome
What are schistocytes
RBC fragments, seen in microangiopathic haemolytic anaemia
What are acanthocytes
Spiculated RBCs seen in liver disease, renal disease and splenectomy
What are stomatocytes
Slit shaped central pallow in RBC, seen in inherited red cell membrane defects and alcoholism
What are target cells
RBC with central pallor that has dense center (bulls eye). Seen in thalassaemia, iron deficiency, cholestatic liver disease
What is a Roleaux formation
Stacked coin agglutination. Abnormal serum protein levels (eg. multiple myeloma)
What is a Heinz body
Denatured, agglutinated haemoglobin in G6PD
What is a degmacyte
Bite cell. Removal of Heinz body
What is a dohle body
Mutliple toxic granules in neutrophils indicating severe trauma or infection (eg. sepsis)
Microangiopathic haemolytic anaemias
Group of haemolytic disorders characterised by
- anaemia
- schistocytes
Haemolytic uraemia syndrome
- triad of
- > microangiopathic anaemia
- > thrombocytopaenia
- > AKI
- usually associated with diarrhoea
- > shiga producing E coli
Thrombotic thrombocytopaenic purpura
- pentad of
- > microangiopathic anaemia
- > purpura
- > AKI
- > neurological signs
- > fever
- caused by
- > deficiency in ADAMTS-13
- > vWF multimeres not cleaved causing platelet clumping
Disseminated intravascular coagulation
- acquired disorder
- > trauma
- > sepsis and infection
- > malignancy
- > toxic reactions
- activation of coagulation pathways
- > intravascular thrombi
- > consumption of platelets and clotting factors
- presents with
- > trigger
- > bleeding and purpura
- > thrombocytopaenia
- > prolonged aPTT/PT
- > low fibrinogen
- > high d-dimer/fibrin degradation production
