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R3 Medicine
Kannadit Prayongratana, M.D.
Anemia
The condition in which the hemoglobin concentration
is below the normal value
WHOs normal Hb/Hct value
Child -4 yr 11.0 33
Children 5-7 years 11.5 35
Children 8-11 years 12.0 36
Female 12.0 36
Male 13.0 40
Sign and symptoms of anemia
Related to anemia
Pallor
Palpitation, tachycardia
Dyspnea, congestive heart failure
Fainting, syncope
Orthostatic hypotension
Shock
Sign and symptoms of anemia
Related to underlying causes
GA : Jaundice, edema
Skin : Rash, oral ulcer, koilonychia, premature
grey hair
Gastrointestinal : Glossitis, odynophagia, Pica,
dyspepsia, melena, hematochezia,
organomegaly, mass lesion
Classification of anemia
Onset : Acute, Subacute, Chronic
Red cell size :
Microcytic anemia (MCV<80 fl)
Normocytic anemia (MCV 80-100 fl)
Macrocytic anemia (MCV >100 fl)
Causes : Hypoproliferative, overutilization
Classification by size
Thalassemia, Hemoglobinopathies 40
Iron deficiency anemia
Sideroblastic anemia
Microcytic
Lead poisoning 60
HS
Anemia of chronic inflammation
Anemia of chronic kidney disease 80
G-6-PD deficiency
Normo-
Aplastic anemia
cytic
Reticulocytosis
Autoimmune hemolytic anemia 100
Macro-
Liver disease
cytic
MDS, Megaloblastic anemia
120
Hypoproliferative anemia
Marrow erythroid hypoproliferation
Pure red cell aplasia
Aplastic anemia
Iron deficency and anemia of inflammation
Bone marrow suppressant : drugs, radiations
Infiltrative marrow : tumors, infections
Myelofibrosis
Ineffective hematopoiesis
Hypoproliferative anemia
Marrow erythroid hypoproliferation
Ineffective hematopoiesis
Megaloblastic anemia
Sideroblastic anemia
Myelodysplastic syndrome
Overutilization anemia
Blood loss
Hemolysis
Intracorpuscular defects
Hereditary:
Membrane defects: HS, HE, SAO, HPP
Enzymopathy: G-6-PD, Pyruvate kinase
deficiency
Acquired : Paroxysmal nocturnal hemoglobinuria
Extracorpuscular defects
Overutilization anemia
Blood loss
Hemolysis
Intracorpuscular defects
Extracorpuscular defects
Immune mediated: AIHA, HDN, HTR
Infections: malaria, clostridial infection
Mechanical: TTP, HUS, Cardiac hemolysis
Drugs or chemical: Pb poisoning
Tools for classification
Reticulocyte count
Corrected reticulocyte count (%)
= Reticulocyte count x Hct
45
Reticulocyte production index (RPI)
= Corrected reticulocyte count
Maturation time
Hematocrit Correction factor
(Maturation days)
39 - 44.1 1.0
34 38.1 1.5
24 33.1 2.0
15 23.1 2.5
<15 3.0
Temple of Poseidon, Sunion, Greece
Pure red cell aplasia
Characterized by
Anemia; NCNC
Reticulocytopenia (<1%, typical<0.1%)
Absent or rare erythroid precursor cells in
the bone marrow (marrow erythroid<5%)
Classification of PRCA
Self-limited
Transient erythroblastopenia of childhood
Transient aplastic crisis of hemolysis (acute B19 parvovirus
infection)
Fetal red blood cell aplasia
Nonimmune hydrops fetalis (in utero B19 parvovirus infection)
Hereditary pure red cell aplasia
Congenital pure red cell aplasia Virus
(Diamond-Blackfan syndrome) Chronic Parvovirus B-19, hepatitis,
Acquired pure red cell aplasia HTLV, EBV
Thymoma and malignancy Pregnancy
Lymphoid malignancies Drugs
(and more rarely other Phenytoin
hematologic diseases) Azathioprine
Paraneoplastic to solid tumors Chloramphenicol
Connective tissue disorders with Procaineamide
immunologic abnormalities Isoniazid
SLE, JRA, RA,PGA Idiopathic
Parvovirus B19
Underlying RBC survival Ability to Outcome Inves-
clear tigation
infection
SAA Non-SAA
age Supportive treatment
BJH,1983 70 100:0 83 28 36 25
Hemonc,1985 72 - 93 25 31 18
BJH,1985 73 51:49 67 38 39 -
AJH,1984 59 65:35 - - - -
Blood,1989 68 53:47 82 45 24 12
BJH,1985 62 58:42 45 32 24 -
BJH,1987 73 50:50 85 - - -
Cancer,1985 68 52:48 - - - 12
Cancer,1983 - 54:46 - - - 21
Diagnosis
CBC: anemia, thrombocytopenia, leukopenia
PBS: present of dysplastic features.
LDH, Indirect hyperbilirubinemia, AST
BM: dysplastic feature, elevated blast but less
than 20%.
BM Prussian blue: ringed sideroblast.
Hematologic findings
RED CELLS
Macrocytosis
Dimorphism
Punctate basophilia
Nucleated red cell
Polychromatophilia
Erythroblast
Hematologic findings
WHITE CELLS
Hypogranulation
Pseudo Pelger-Huet anomaly
Hypersegmentation
Ringed Neutrophil
Dohle bodies
Myeloblast
Immature cells
Hematologic findings
PLATELETS
Giant platelets
Hypogranulation
Megakaryocyte fragments
Bone marrow findings
CELLULARITY
Increased or normal cellularity is the most
common finding (81-100%) supporting ineffective
hematopoiesis.
Hypocellular marrow
found in 0-19% without
significance
(clinical & prognosis)
Fibrosis 20-25%
Bone marrow findings
DYSERTYTHROPOIESIS 73-100%
Multinuclearity
Intranuclear bridging
Nuclear fragment and budding
Megaloblastic change
Vacuolation
Ringed sideroblast
Maturation arrest
Bone marrow findings
DYSERTYTHROPOIESIS
Bone marrow Prussian blue
RINGED SIDEROBLAST
Abnormal iron storage in mitochondria of
erythroid around the nucleus.
Defined as 1/3 or more erythroid nucleus is
encircled by 10 or more siderotic granules.
Bone marrow pathology
RINGED SIDEROBLAST
Bone marrow findings
DYSMYELOPOIESIS 37-88%
Giant primary granules.
Decreased primary granules.
Auer rods.
Decreased secondary granules.
Fragmented or abnormal nuclei.
Maturation arrest.
Abnormal localization of immature precursors
(ALIPS).
Bone marrow findings
DYSMYELOPOIESIS
Bone marrow pathology
Abnormal localization of immature precursors
(ALIPS)
Bone marrow pathology
Abnormal localization of immature precursors
(ALIPS)
The anatomic interrelations between
hematopoietic precursors and the bone marrow
stroma.
Interface between marrow and bone, may be
disordered in patients with MDS.
Such relations regulate the normal
hematopoiesis.
Bone marrow pathology
Abnormal localization of immature precursors
(ALIPS)
Stromal cells are important source of various
growth and inhibitory factors.
Specific binding to stromal matrix proteins may
also control the local concentration of growth
factors.
Bone marrow pathology
Abnormal localization of immature precursors
(ALIPS)
Myeloblasts and other immature forms are
normally localized along the endosteal surface.
In many patients they form clusters (ALIPs)
that are not contiguous with bony trabeculae.
Bone marrow pathology
DYSMEGAKARYOPOIESIS 50-87%
Micromegakaryocytes
Decreased nuclear polyploidy
Separated nuclei
Le Chteau de Chantilly, France
Vardiman JW : Blood 2002 :100(7) :2297
FAB and WHO
FAB WHO
RA RA
5q- syndrome
RCMD
RARS RARS
RCMD/RS
RAEB RAEB-1
RAEB-2
RAEB-t AML
CMML MDS/MPD
Impact of WHO classification
control
patient
HS: Therapy
In mild case therapy may not required.
Folate supplement is necessary in moderate to
severe case
Splenectomy is the definite treatment but this
must pay cost of infectious risk
Indications:
Symptomatic gallstone (with cholecystectomy)
Frequent hemolytic crisis
Failure to thrives
Patient desire
HS: Therapy
Special considerations:
Presplenectomize vaccination
at least 2 weeks before splenectomy.
Polyvalent pneumococcal vaccine
Hib vaccine
Meningococcal vaccine
Postsplenectomy prophylactic antibiotics
for 2-5 years
HS: Therapy
Kinderdijk, Netherlands
Tutorial for my friends:
R3 Medicine
Kannadit Prayongratana, M.D.