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Erythrocytosis
Too many RBCs
  • Polycythemia Vera
  • Secondary Polycythemia: Appropriate, Inappropriate
  • Relative Polycythemia: Dehydration, Spurious
Anemia
Too few RBCs
  • Relative
  • Decreased production
  • Increased Destruction
Relative Anemia
  • not "true" anemia
  • Have a normal amount of red blood cells in the body, but too few in circulation
  • Hypersplenism: RBCs sequestered in spleen
  • Pregnancy: increased plasma volume
Anemia caused by decreased production
LOW rectiulocyte count
elements to make RBCs are missing
  • Aplastic Anemia
  • B-12, Folate, Iron Def.
  • Chronic disease & inflamm.
  • Endocrine Disease
  • Marrow infiltration
  • Pure Red Cell Aplasia
  • Renal Disease
  • Sideroblastic Anemia
Anemia caused by increased destruction
  • Abnormal Hb: Qual & Quant
  • Enzyme defects
  • Autoimmune hemolytic anemia
  • Mechanical
  • Membrane defects
  • Burns, Chemicals, MOs, Paroxysmal Nocturnal Hburia, porphyria
Abnormal Hemobglobin Anemias
  • Increased Destruction
Qualitative
  • Sickle Cell Anemia
  • Hemoglobin C
  • Unstable Hemoglobin
Quantitative
  • Alpha & Beta Thalassemia
Enzyme Defect causes of Anemia
  • Increased Destruction
  • G6PD deficiency
  • PK deficiency
Autoimmune Mediated causes of Anemia
  • Increased Destruction
  • Warm antibody
  • Cold Antibody
  • Drug Related
  • HDN: hemolytic disease of the newborn
Mechanical causes of Anemia
  • Increased destruction
  • Microangiopathic
  • March Hemoglobinuria
  • Traumatic Heart Valve
Membrane Defects that cause Anemia
  • Increased destruction
  • Hereditary spherocytosis
  • Ellopocytosis
  • Acanthocytosis
Myelo-
all formed elements of the blood: RBC, WBC, platelets
Thrombo-
Platelets
Reticulo-
Early RBCs
Schisto-
fragmented/hemolysed cells
Poiklio-
different shapes & sizes
-blastic
early cell
-cytic
cell in general
-ilia
too many
Shifts in Hematopoietic Developmnet: Shift to the LEFT
shift towards making more immature cells (blast cells)
**How to Evaluate Anemia: Initial
  • Excellent medical history
  • Good physical exam: hypersplenism, conjunctiva, jaundice, lymphadenopathy
  • Visual inspection of blood smear: will see schistocytes
  • CBC
  • Indices
  • Reticulocyte Count
**Complete Blood Count (CBC)
  • Hematocrit (HCT)
  • Hemoglobin (Hgb)
  • Red blood cell count (RBC)
  • Red blood cell indices
  • WBC count
  • Platelet count
  • Blood smear
**RBC Indices
MCV: Avg. RBC size

MCH:
Hemoglobin amt. per RBC

MCHC:
amt of Hgb relative to the size of the cell (Hgb []) per RBC
How to Evaluate Anemia: As Indicated
  • Reticulocyte count
  • Bone marrow aspiration
  • Iron/TIBC
  • B12 level
  • Folate level
  • Osmotic Fragility Test
  • DAT/IAT
  • Billirubin
  • Hgb electrophoresis
ONLY WHEN NEEDED!
**Clinical Changes in Hemolysis
  • + IAT: Ab in plasma
  • + DAT: Ab coated on RBCs--> destruction & hemolysis
Free Hgb causes:
  • Hgbemia
  • Billirubinemia
  • Low serum haptoglobin
  • Hemoglobinuria
Stroma (RBC membrane & parts) causes:
  • Renal shutdown
  • IC: DIC--> bleeds
Urine Sampling and Anemia
When urine sits:hematuria (kidney disease)--> Hgburia (anemia)

This is why you must look @ a fresh urine sample
Thrombotic Thrombocytopenic Purpura (TTP)
  • disease that leads to blood clots in small vessels
  • Histology: schistocytes & thrombocytopenia (no platelets)
Sickle RBCs: Histology
  • Change in Hgb Shape
  • Biconcave disc becomes sickled & gets stuck in microvasculature
Autoimmune Hemolysis: Histology
Spherocytes
  • extravascular, in spleen
  • RBC w/ Ab on surface goes to spleen
  • spleen removes Ab & part of RBC membrane
  • Shape: biconcave--> sphere (decreases surface area)
Alternative Classification of Anemia: Based on Morphology (RBC indices)
  • Microcytic Hypochromic
  • Normocytic Normochromic
  • Macrocytic
  • Combination of partial
Microcytic Hypochromic
  • Chronic Disease
  • Hemoglobinopathy
  • Iron deficiency (seek cause)
  • Sideroblastic (80%)
  • Thalassemia
Macrocytic: High & Low Reticulocyte Count
Low Reticulocyte Count
  • B-12 & Folate Deficiency
  • Pernicious anemia
  • GI-disease, malabsorption
  • Disease of DNA synthesis
  • Liver Disease
  • Sideroblastic Anemia 20%
High Reyiculocyte Count
  • Prior hemolysis/hemorrhage
  • Treated B-12 or Folate deficiency
Normocytic Normochromic
Normal Looking
  • Myelofibrosis
  • Sickle cell
  • Acute blood loss
  • Aplastic anemia
  • Cancer
  • Granulomas
  • Marrow Invasion
  • Remote effects
  • Chronic disease
  • Chronic Infx & Inflamm.
  • Hemolysis:many
Normocytic Normochormic: Hemolysis
  • burns
  • DIC
  • drugs
  • Enzyme defect
  • Hgbopathies (HgbC, Thalassemia)
  • Hereditary spherocytosis
  • Hypersplenism
  • Autoimmune
  • PNH
  • TTP
  • Prostethtic heart valve damage
  • Vasculitis
Normal Platelet Development
Megakaryocyte to Platelet

Qualitative & Quantitative: ex. may have low platelets but they are large & have increased fxn.  OR you may have a normal amount of platelets, but they have a decreased fxn. 
  • Normal Size
  • Normal Function
How to Evaluate Platelet Disorders
Excellent medical history
Good physical exam: petechia, ecchymosis, conjunctiva
Visual inspection
of blood smear
CBC w/ platelet count

If indicated: Bone Marrow exam
Petechiae
on skin, result of too few platelets

RBCs (instead of platelets) fill in between cells @ basement membrane = Petechiae
non-blanchable
form on dependant body part: Ex. Running: on leg, Bed Rest: back/buttocks
Disorders of Platelets
  • Qualitative
  • Quantitative: Thrombocytopenia (too few) or Thrombocytosis (too many)
Qualitative d/o of Platelets
Acquired
  • Drug induced
  • Myeloproliferative d/o
  • Uremia
Congenital (think Kids)
  • Bernard Soulier's Disease
  • Glansman Disease
  • Storage Pool Disease
Drug Induced Qualitative Platelet d/o
Ex. Aspririn

Aspirin exposed platelets
  • do NOT adhere
  • DO aggregate
  • need 1/3 of platelets to be unexposed to aspirin
  • usually takes 3 days
Quantitative Thrombocytopenia
Decreased Production
Dilution
Distribution
Increased Destruction
QuantitativeThrombocytopenia: Decreased Production of Platelets
Aplastic Anemia
Congenital
Drug induced
Marrow infiltration
Nutritional
Post Chemo & Radiation
PNH
Viral
Quantitative Thrombocytopenia: Dilution
  • Massive blood transfusions
  • Pregnancy (+plasma volume)
Quantitative Thrombocytopenia: Distribution
normal amount of platelets in body, but they are not available in circulation
  • hypersplenism
  • hypothermia
Quantitative Thrombocytopenia: Increased Destruction
Congenital: Iso immune neonatal, HDN, Maternal idiopathic TP

DIC: consumptive coagulopathy
Drug induced
Hemolytic Uremic Syndrome
Idiopathic Thrombocytopenic Purpura
Infection
Post transfusion Purpura
Thrombotic Thrmobocytopenic Purpura (TTP)
Traumatic (extracorporeal circ)
Thrombocytosis
Acute hemorrhage
Acute & Chronic inflammation
Hemolytic anemia
Post splenectomy
Myeloproliferative d/o: CML, Erythroblastic Leukemia, Hyper-eosinophilia syndrome, 1° Thrombocythemia, Myelofibrosis w/ myeloid metaplasia, Polycythemia Vera
Naming based on presentation of -cytosis
Too many platelets: 1º thrombocytosis

Too many RBC: Polycythemia Vera

Too many WBC: CML
White Blood Cell d/o
  • Acute Leukemia
  • Chronic Leukemia
  • Lymphoma
  • Myelodysplastic syndromes
How to evaluate WBC d/o
H&P
CBC w/ WBC differential
Lymph Node evaluations:
radiological, histological, immuno-oncologic

Bone marrow aspirate & Biopsy:
2º, definitive

*not all lymph nodes should be biopsied, base of physical exam
Myeloid vs. Lymphoid
Myeloid: from bone marrow, precursors of granulocytes (PMNs, basophils, eosinophils), monocytes, RBCs, platelets (all formed elements except lymphocytes)

Lymphoid: from thymus, lymph nodes, spleed, includes lymphocytes from the peripheral blood, lymph nodes & extranodal tissue
Acute vs. Chronic Leukemias
Acute
  • aggressive (months) course
  • BLASTS predominate
  • acute lymphoblastic leukemia
Chronic
  • slow (years) course
  • mature malignant cells
  • chronic lymphocytic leukemia
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