Anaemia - WordPress.com

advertisement
Anaemia
Cause
MICROCYTIC
Anaemia
MCV <80
Incr RDW
Type
Cause
GI / GU blood loss; Malabsorption
Pregnancy; Dietary
Sx / Mng
Mng: IV Fe if severe anaemia not
requiring b/ transfusion, or unable to
tolerate PO
(may be
normo)
Normal globin chains produced at decreased
rate  haemolysis (hence normo)
Alpha: more common in SE Asia; HbBarts (4;
IU demise); HbH (3; mod disease); trait (2;
asymptomatic)
Beta: more common in Europe; Hetero =
minor (asymptomatic); homo = major; more
symptomatic than alpha
Fe overload (hepatosplenomegaly,
cardiac disease)
Pathological #, bone deformities,
paraspinal masses
Endocrine failure
Recurrent infections
Mng: trt Fe overload with
desferrioxamine; stem cell transplant;
often require transfusions
Decr: Ferritin (<15), Fe (<13), Hct (<30,
occurs late)
MCV:Hb 10:1
Incr: reticulocytes, erythroblasts
(haemolysis)
HbF (>90%)
Normal: RBC count
Decr: MCH; Hb (3-9)
Poikilocytosis, anisocytosis, incr no. of
erythroid precursors on BM biopsy,
nucleated RBC’s, basophilic stippling, target
cells
Mng: Pyridoxine trial 2-3/12
Incr: Fe + Ferritin (may be normal)
Sideroblastic
Hereditary (may respond to pyridoxine)
Lead poisoning, ETOH
Myelodysplastic syndrome /
myeloproliferative disease
Fe def
Thalassaemia
MACROCYCTIC
Anaemia
MCV
>100
Vit B12
def
MCV >115
Folate
def
MCV >115
Other
Incr: TIBC, transferrin (>3.6)
Normal: Fe binding capacity
Sideroblasts in BM
Incr: globulins
Multiple myeloma
AOCD
Blood findings
Hypothyroidism; vit C def
Pernicious anaemia (autoab to parietal cell
and intrinsic factor  decr production of IF,
prevent formation of complex; mean onset
60yrs)
Gastrectomy, gastric atrophy, ileal disease,
pancreatic disease
Malabsorption (most common cause;
eg. Bacterial overgrowth)
Dietary (ie. Vegans)
NO (prolonged / repeated exposure)
Assoc with: autoimmune thyroiditis, Graves,
Addisons, 1Y hypoPT, vitiligo, MG
Dementia, psychosis, peri neuropathy,
subacute degeneration of spinal cord
(occurs faster if only folate
supplementation; due to
demyelination; affects post + lat
columns), abnormal gait
Gastric adenoCa / carcinoid
Achlorhydria, painful smooth tongue
Dietary (most common; esp in
alcoholics)
Malabsorption
Incr demand (pregnancy, malignancy,
psoriasis)
Dihydrofolate reductase inhibitors (eg.
methotrexate, trimethoprim)
Phenytoin, Na valproate
Mng: 5mg folate OD; vit B12 if def
suspected
Normal: ferritin
Decr: RBC count
Incr: LDH
Decr: B12; WBC and plt;
holotranscolabalmin II
Anti-parietal cell and IF ab’s;
Hypersegmented neutrophils; Stippling of
red cells; Howell Jolly bodies; Schilling test
(decr urinary B12 that is incr by giving IF)
Mng: Vit B12 injections; high dose PO
vit B12
Incr: RDW (increases early, and shows there
is a variety in size of RBC’s)
Decr: folate
Alcoholism; chronic liver disease; hypothyroidism; congenital cyanotic heart disease; myelodysplastic syndromes; Megaloblastic
reticulocytosis
NORMOchromic
Anaemia
MCV 80100
Aplastic
anaemia
(becomes
macrocytic)
AOCD
(becomes
macrocytic)
Esp in SE Asia; cause by stem cell loss
Idiopathic (50-65%)
Fanconi’s syndrome (autosomal recessive)
Radiation
Chloramphenicol, gold, sulphur drugs,
chlorpromazine, streptomycin
Viral infections (eg. hepatitis)
SLE; Pregnancy; Graft vs host
Decr tissue oxygenation,
immunocompromise, bleeding
Fatigue, weakness
No splenomegaly
Most common cause of normocytic
Mng: b/ transfusion; consider Fe;
erythropoietin
Due to incr hepcidin, decr erythropoietin,
inhibited marrow proliferation, altered Fe
distribution, decr RBC life span, incr reticuloendothelial activity
RA, CRF, Ca, CT disorders
Defect in membrane proteins (spectrin) 
decr deformability  haemolysis
Hypocellular BM
Mng: supportive; ABx; blood products;
immunosupp; G-CSF
ARF
Spherocytosis
Decr: reticulocytes, WBC, plt
Usually asymptomatic
Splenomegaly (moderate; 50% young
children, 85% adults)
Jaundice, gallstones (5% children, 50%
adults)
Haemolytic crises (usually during
infections; mild)
Aplastic crises (parvovirus infection)
Leg ulcers, spinal cord lesions
Mng: splenectomy, cholecystetcomy
Incr: ferritin
Normal: reticulocytes and RDW
Decr: Fe, transferrin, TIBC
Normal reticulocytes and RDW
Features of haemolysis +
Incr: RDW
Decr: MCHC (in 50%)
Spherocytes (quite non-specific); incr
osmotic fragility; variable degree of anaemia
Coombs negative
Haemolysis screen = haptoglobins,
LDH, Coombs, unconj/conj bil
Urinary: urobilinogen
Elliptocytosis
 haemolysis
Similar to above but milder
As per spherocytosis
G6PD def
X-linked inherited; more common in males,
Black
Splenomegaly, neonatal jaundice,
gallstones
Features of haemolysis +
G6PD assay
Due to oxidative stress  haemolysis
Trigger: infection; sulphonamides,
nitrofurantoin, antimalarials, vit K, aspirin
PK def
Sickle cell
disease
Autosomal recessive 
haemolysis
Autosomal recessive; account for 70%
congenital Hb disorders; esp in Black (occurs
in 8%), Middle East, Indian
Defect in Hb production (valine/glycine) 
HbS  polymerization when low NO conc
 damage to membrane 
haemolysis, incr intracellular Ca, rigid
RBC’s, activation of thrombosis
Triggers: hypoxia, acidosis, 2,3,DPG,
vascular stasis, IV vol depletion, infection,
dehydration, altitude, cold
Acquired
autoimmune
haemolytic,
cold
Extravascular haemolysis via type II
hypersensitivity reaction: autoab vs RBC
 IgM on RBC in peripheries
 destroyed in reticuloendothelial
system
Lymphoproliferative, infections (eg.
Mycoplasma)
Mng: partial response to splenectomy
Features of haemolysis
Onset after 6m (when HbF gone)
Arthralgia, dactylitis (most common
presentation <2yrs; periosteal elevation
on XR, painful swelling); bone thinning
CCF; murmurs in 80%
Jaundice, gallstones, AP,
Hepatosplenomegaly (spleen small in
adulthood due to recurrent infarction;
functional asplenia); incr bil; jaundice;
gallstones
Leg ulcers
Sepsis is most common cause of death
in children, esp strep pneumoniae
Vasoocclusive crises (MS, GI, RS, CNS,
renal), CVA, pul infarction; acute chest
syndrome
Haematologic crises (splenic
sequestration – 2nd most common cause
of death, aplastic, infectious)
Splenomegaly
Reynaud’s disease and other symptoms
of circulatory obstruction
Livedo reticularis
Worse in winter
Features of haemolysis +
Incr: LFT’s, plt
Hb 6-9; HbF 2-20%; RBC survival 10-20/7
(instead of 120); reticulocytes 5-15%
Haematuria; Sickle cells; Howell Jolly bodies
For Mng: see Dunn
Features of haemolysis +
Incr: plt, WBC
Haemaglobinuria
Coombs positive
More common than cold
Splenomegaly
Features of haemolysis +
Acquired
autoimmune
haemolytic,
warm
Extravascular haemolysis via type II
Microangiopathic
haemolytic
anaemia
Intravascular haemolysis
Features of haemolysis +
DIC, TTP, HUS, prosthetic heart valves, malignant HTN, pre-eclampsia, Wegener’s
granulomatosis, malignancy, HELLP, snake envenoming, vasculitis
Irregularly fragmented RBC’s, Helmet cells
RBC
mechanical
trauma
Intravascular haemolysis (eg. March Hburia, cardiac valve, IABP)
Features of haemolysis
Other causes of
Infections: Clostridia, CMV, coxsackie, EBV, haemophilus, herpes simplex, HIV, malaria,
measles, mycoplasma, varicella, IMN
Drugs: antimalarials, arsenic, bites, copper, lead, LA, nitrates, sulfonamides, ceftriaxone
Autoimmune: incompatible b/ transfusion, SLE, RA, UC, hepatitis, lymphoma, CLL
Haemolytic disease of the newborn: Rh of mother crosses placenta; need anti-D
Features of haemolysis:
haemolysis
hypersensitivity reaction: autoab vs RBC
 IgG / complement or both on RBC
 destroyed in reticuloendothelial
system
50% idiopathic; 50% 2Y
(lymphoproliferative, post-infection,
autoimmune, neoplasm, CT disorder, drugs
(eg. Methyldopa, penicillin))
Paroxysmal
nocturnal
haemoglobinuria
Intravascular haemolysis
Others
BM infiltration, congenital (eg. Fanconi’s
syndrome, Diamond-Blackfan syndrome)
Def of membrane proteins  injury to BM
stem cell pool  autoimmune lysis of RBC,
WBC + plt; functional WBC defect
Incr: plt, WBC
Coombs positive
Predisposition to thrombosis (venous
thrombosis in 20%, often prox)
5% become AML
Mng: immunosupp; BM transplant may
be curative
Incr: LDH, unconjugated bil, reticuloctyes,
mild MCV, RDW, urobilinogen, faecal
stercobilinogen
Decr: haptoglobin
Heinz bodies
In intravascular: methaemalbumin
Features of haemolysis +
Decr: WBC, plt, Fe
Haemoglobinuria, urinary Fe; Acidosis
Download