Normocytic anemia Flashcards
Anemia considerations -
Rate of H/H alteration (slow vs fast) evidence of - Jaundice, scleral icterus - Hepatosplenomegaly - ^-retics - v-haptoglobin (reclaiming hgb) -Spherocytes/schistocytes
DDx of normocytic anemia
Anemia of chronic dz Acute blood loss Multifactorial anemia Renal-failure ass/w anemia Aplastic anemia Dilutional anemia (in-pt) Hemolysis
2nd MC anemia (behind IDA)
Anemia of chronic disease
Anemia of chronic disease has what morphology of RBCs?
Normochromic, normocytic
Underlying causes of Anemia of chronic disease?
Dilutional anemia (25% due to IVF inpt) Systemic inflammatory process (SLE, RA, Malig)
Anemia of chronic disease pathophys
Inflam process disrupts iron hemostasis
IL-6 stimulates hepcidin release from liver
Decrease iron in BM > Erythropoiesis reduction
+
Systemic inflam destroying RBC’s (v-Survival)
Anemia of chronic disease TXT
UC Iron replacement Blood transfusion PRN EPO if - anemia is symptomatic + Hgb <10g/dL OR - Cancer w/ active chemo - CKD (GFR <30mL/min) - HIV txt w/ evidence of myelosupression
Acute blood loss occult reasons?
GI bleed
Retroperitoneal bleed (S/P Cardiac cath)
Post-op bleeding
Acute blood loss RBC morpholoy is initially? But evolves into?
Normocytic > microcytic
CKD anemia GFR?
<30mL/min
CKD - etiology of the anemia?
Decrease EPO > decreased BM production of RBCs
CKD anemia management
EPO bld measurement - not req (PRN)
TXT - synthetic EPO stimulating agents when
-1. GFR <30mL/m AND -2. hgb <10g/dL
CKD anemia TXT goal
Hgb - 11g/dL
HCT - 33%
Primary Aplastic anemia is?
IgG autoantibody against BM stem cells
Secondary Aplastic anemia is due to?
Inheritied/acquired
- Chemo/Rad
- Rx
- Infections
Pancytopenia BM D/O
Aplastic anemia MDS Acute leukemia Myelofibrosis Infiltrative D/O (lymphoma, myeloma, carcinoma) Megaloblastic anemia
Pancytopenia Non-Clonal (Non-BM) D/O
Hypersplenism
SLE
Aplastic anemia clinical features
Hallmark is Pancytopenia (+hypocellular BM)
Anemia - weak, fatigue, pallor (Usually very severe)
Neutropenia - high risk of infection
Thrombocytopenia - bleeading
Aplastic anemia population age group
Bimodal
15-25yo
AND
>60yo
Aplastic anemia TXT
BM transplant - younger and/or better fx status
Immunosupression - Older patients (graft vs Host dz)
Case by case however
Hemolytic anemia general attributes
Continuous or intermittent
Intrinsic or Extrinsic
Intrinsic Hemolytic anemia is due to?
Defect in the RBC itself (usually hereditary)
- membrane defect = hereditary spherocytosis
- oxidation vulnerability = G6PD
- hemoglobinpathy = SCA or Thalassemia
Extrinsic Hemolytic anemia is due to?
Immune - (autoimmune, lymphoproliferative, Rx toxicity)
Microangiopathic - TTP, HUS, valve hemolysis
Infection - plasmodium, clostridium, borrelia
Burns
Hemolytic anemia smear findings
Schistocytosis (req’d for hemolysis to be Dx)
Hemolytic anemia labs
Indirect bili - INC - esp if intravascular hemolysis
Hemoglobinuria - INC - telltale intravascualr hemolysis
LDH - INC - released from lysed RBCs
Retic - INC - (however, Low-NL if + BM D/O)
Haptoglobin - DEC - binds free Hgb as RBCs lyse
MC intrinsic hemolytic anemia?
Hereditary Spherocytosis
Hereditary Spherocytosis is due to?
ABNL spectrin or actin (proteins that provide membrane structure) thus become trapped in spleen and rupture. (Chronic hemolysis)
Hereditary Spherocytosis have RBCs that cannot?
Pass through small fenestrations of the spleen due to lack of plaibility (Increased fragility)
Hereditary Spherocytosis RBCs appear on smear?
Normocytic anemia - Round RBCs, lack concavity, central pallor
Hereditary Spherocytosis pop predisposition?
Autosomal dominant D/O
MC Dx in childhoos (can be adult)
Important Hereditary Spherocytosis Labs
MCHC is increased (rounded shape increases surface area and allows more room for Hgb) Reticulocytosis Spherocytosis Negative Coombs test = (not autoimmune) Increased osmotic fragility test
Hereditary Spherocytosis TXT
Supportive
pRBCs if Hgb <7g/dL
Folic acid supplement (required for Erythropoiesis)
Splenectomy (rare - eliminates hemolysis site)
MC enzymatic RBC D/O?
G6PD deficit
G6PD deficit causes?
Hemolysis if RBCs are exposed to oxidative stress.
G6PD deficit genetic predisposition?
X-linked recessive
Southeast asia, Mediterranean, African American
G6PD deficit pathophy
NL G6PD acts on glutathione in a reduced state which protects Hgb from oxidative denaturation
Oxidative stress is?
bodys inability to detoxify oxygen free radicals > thus they attack biological molecules (DNA, lipids, Proteins)
Oxidative stress and a pt w/ G6PD deficit causes
Episodic hemolysis in relation to the insult
Oxidized stress onto Hgb causes what to happen?
Denatures into Heinz Bodies (inclusion w/in RBCs)
Rx that induce oxidative stress?
Antimalarials - (Primaquine, Dapsone)
Sulfonamides - (Sulfonamethoxazole)
G6PD deficit clinical course?
Pt is perfectly healthy unless exposed to oxidative stress
- Rx
- Dehydration
- Hypoxia
G6PD deficit labs to focus on
Retics - INC Indirect bilirubin - INC LDH - INC Haptoglobin - DEC Blood smear - Heinz bodies (denatured hgb)
SCA genetic predispostion
Autosomal recessive D/O
African American
Usually Dx 1st yr life (Hgb F stops revealing Hgb S)
SCA mutation is?
Point mutation in Beta chain at 6th position resulting in the formation of Hgb-S > sickling RBCs
ABNL hgb shape causes?
Chronic hemolytix Anemia
Acute painful episodes due to vaso-occlusion
Acute vaso-occlusions w/ SCA occur when?
Spontaneously OR Oxidative stress (Inf, Dehydrated, hypoxia)
Vaso-occlusions are a risk factor for?
Stroke
Acute manisfestations of vaso-occlusions
Bacterial Sepsis/meningitis Recurrent Pain - dactylitis, MSK, Abd pain Stroke Splenic sequestration Aplastic crisis Priapism Hematuria (including papillary necrosis)
Chronic manifestations of vaso-occlusions
Anemia Jaundice Splenomegaly Fx asplenia \+ others
SCA features?
Chronic hemolysis
Smear = Sickles and Howell-Jolly bodies (nuclear remnants - usually removed by spleen)
Fx hyposplenism/Asplenia
SCA Dx is confirmed how?
Hgb electrophoresis
SCA TXT
Hydroxyurea - INC Hgb F (if >2 acute painful crises/yr)
Pneumococcal Vaccine - encapsulated bugs - asplenia
Transfusion therapy - Hgb <7.0g/dL
IVF - NS or LR
Pain - ( 1-3 APAP, 4-7 NSAIDS, 8-10, (hydro)-morphine
Autoimmune hemolytic anemia (AIHA) is?
acquired autoantibodies to RBCs membranes
AIHA important features
Episodic hemolysis trigger w/ physiologic stress
Rapid onset anemia - possibly life threatening
+ Coombs DAT (checks for antihuman antibodies)
Purpose of a Coombs direct antiglobulin test
Checks for antihuman antibodies in the blood
Microangiopathic Hemolytic anemia (MAHA) is?
group of D/O w/ intravascular hemolysis due to RBC fragmentation
Hallmark S/S of MAHA is?
Schistocytes