Pathophys: Hematology Flashcards
hemoglobinopathy
group of disorders (inherited) = abnormal production or structure of hemoglobin molecule (e.g., sickle cell anemia)
coagulopathy
a disease/condition affecting the bloods ability to coagulate
aplasia
incomplete, retarded or defective development of tissue/organ; cessation of the usual regenerative process in a tissue/organ [aplastic anemia: body stops producing new blood cells]
anisocytosis
a variation in size of cells (esp RBCs, pernicious anemia)
anemia
hemoglobin concentration is below normal bc of a deficiency in RBCs and/or a low level of hemoglobin in cells
hemolysis/hemolytic
break down of RBCs
polycythemia
disorder of bone marrow (resulting in over production of RBCs, and a little increase in WBCs and platelets)
cytosis vs. cytopenia
cytois - condition in which there is more than the usual number of cells
cytopenia - deficiency of cellular elements of the blood
poikilocytosis
a condition characterized by poikilocytes in the blood; a poikilocyte is an abnormally shaped RBC
pancytopenia
abnormal reduction in the number of erythrocytes, WBCs and platelets in the blood (all 3 must be low)
mycrocytic anemias (2) (5 questions)
iron deficiency
thalassemia - major/mino
macrocytic anemias (4) (4 questions)
folic acid deficiency
B12 deficiency
liver disease
myelodysplasia
normocytic anemia (1) (4 questions)
anemia of chronic disease
hemolytic anemia (7) (4 questions)
1 sickle cell anemia (Hb S disease) 2 sickle cell trait 3 G6PD deficiency 4 cold agglutinin disease 5 hemoglobin S-C disease 6 hereditary sperocytosis 7 autoimmune hemolytic anemia
what is hemochromatosis?
1 question
disease in which the body absorbs too MUCH iron from diet; iron is stored in liver and pancreas and can be toxic leading to cancer, heart arrhythmias and cirrhosis
bone marrow failure diseases (3)
1 question
aplastic anemia
pure red cell aplasia
myelodysplasia
leukemias and myeloproliferative disorders (7)
6 questions
- acute lymphocytic leukemia (ALL)
- chronic lymphocytic leukemia (CLL)
- polycythemia vera
- myelofibrosis
- acute myelogenous leukemia (AML)
- chronic myelogenous leukemia (CML)
- essential thrombocytosis
lymphomas and plasma cell dyscrasias (4)
6 questions
- hodgkin’s disease
- multiple myeloma
- non-hodgkins lymphoma
- burkitts lymphoma
disorders of hemostasis (5)
3 questions
- immune thrombocytopenic purpura
- thrombotic thrombocytopenic purpura
- hemolytic-uremic syndrome
- henoch-schonlein purpura
- heparin-induced thrombocytopenia
hereditary disorders of coagulation (3)
2 questions
- factor VIII deficiency (hemophilia A)
- factor IX deficiency (hemophilia B)
- von Willebrands disease
acquired disorders of coagulation (4)
2 questions
- vitamin K deficiency
- coagulopathy of liver disease
- disseminated intravascular coagulation (DIC)
- acquired platelet disorders
acquired and congenital hypercoaguable states (6)
2 questions
- factor V Leiden
- malignancy
- activated protein C resistance
- lupus anticoagulant
- protein C deficiency
- protein S deficiency
iron deficiency
- blood loss until proven otherwise
- microcytic
- smooth tongue, brittle nails, koilonychias
thalassemia
- defect making hemoglobin (genetic)
- greater degree of microcytosis
- normal or elevated RBC and retic (unique of the anemias)
folic acid deficiency
- NO NEURO defects
- macrocytic
- glossitis
- pallor
B12 deficiency
- YES NEURO DEFECTS
- macrocytic
- pallor
- glossitis
liver disease
- liver enzymes (which transport B12) not being produced
- B12 not absorbed
- macrocytic
anemia of chronic disease
-chronic disease associated with it
sickle cell anemia
- hemoglobin S, homozygous, hereditary
- recurrent pain
- transfusion, folic acid
sickle cell trait
- hemoglobin S, heterozygous, hereditary
- no tx, genetic counsiling
- increased risk
G6PD deficiency
- enzyme defect
- HEINZ bodies! cause membrane damage
- episodic hemolytic anemia
- avoid antioxidant drugs
cold agglutinin disease
- ACQUIRED
- IgM “cold” agglutinin
- anemia with exposure to cold
- lysis is intravascular
- coombs + test
- tx w/ rituximab
hemoglobin SC disease
- no sickling
- hemoglobin S and C (heterozygous)
- mild course
autoimmune hemolytic anemia
- acquired
- IgG “warm” autoantibody
- rapid onset of life threatening anemia
- spherocytes!
- coombs + test
- tx w/ steroids and maybe splenectomy
aplastic anemia
- hematopoetic stem cell
- bone marrow failure
- pancytopenia
- pupura, petichiae, fatigue, mucosal bleed
- NO ABNORMAL CELL MORPH
- NO LYMPH, LIVER, SPLEEN issues
pure red cell anemia
-autoimmune
-bone marrow failure
-low retic count, normal RBC
diff than aplastic (all cell lines)
diff than myelodysplasia (weird morphology)
myelodysplasia
- acquired
- bone marrow failure
- PRELEUKEMIA
- MCV normal or increased (macrocytic anemia)
- <20% blasts
- MORPHOLOGIC abnormalities
ALL
childhood disease
lymphocytes (b and t cells)
pancytopenia
>20% blasts
AML
adult onset
myeloid cells
pancytopenia
>20% blasts
CLL
B lymphocytes
lymphocytosis (lots!)
no tx unless symptomatic
CML
PHILADELPHIA mutation!
increased WBC count
progress to AML
polycemia vera
increased RBCs (and increased other cells too)
expanded blood volume
thrombosis
increased B12
myelofibrosis
poikilocytosis
weird platelet morphology
other counts variable
essential thrombocytosis
increased platelets
thrombosis
oral hydroxyurea
hodgkins disease
young adult
lymphadenopathy
B cell lymphoma
rare
multiple myeloma
plasma cell cancer
destruction of bone marrow and bone
non hodgkins lymphoma
more common than HL
tumors from lymph tissue
B cell (85%)
burkitts lymphoma
aggressive B cell NHL
rapid growth of tumors and lysis
high risk EBV + malaria
prevalent in children in Africa
immune thrombocytopenic purpura
auto immune disease
destruction of platelets
can be seen w/ lupus, lymphoma, meds, hep C, HIV
“diagnosis of exclusion”
thrombotic thrombocytopenic purpura
thrombocytopenia destruction of platelets fever NEUROLOGIC S/S ADULTS schistocytes
hemolytic-uremic syndrome
thrombocytopenia destruction of platelets KIDNEY DISEASE, DIARRHEA KIDS schistocytes
henoch-schonlein purpura
vasculitis in children (arthritis, abd pain) palpable purpura (extremities, buttocks)
heparin induced thrombocytopenia
BOTH thrombocytopenic and prothrombic state (YAW!)
d/c heparin immediately
factor 8 deficiency (hemophilia A)
hereditary (more common than hemophilia B) elevated PTT (intrinsic) MALES unexpected bleed w/ trauma/invasion joint/soft tissue bleeds
factor 9 deficiency (hemophilia B)
hereditary (less common than hemophilia A) elevated PTT (intrinsic) MALES unexpected bleed w/ trauma/invasion joint/soft tissue bleeds
von willebrands disease
hereditary (most common!) PTT elevated (intrinsic) type 2 like hemophilia A but FEMALES AFFECTED
vitamin K deficiency
acquired
elevated PT (extrinsic)
issue w/ diet/absorption/production
coagulopathy of liver disease
acquired
decrease synthesis of clotting factors and fibrinogen
elevated PT (extrinsic) and PTT in advanced disease
DIC
acquired decreased platelets and fibrinogen elevated PT and PTT secondary disorder bleeding at mult. sites
acquired platelet disorders
acquired
drugs increase thrombin time
heparin has antidote, newer drugs dont!
activated protein C resistance
hereditary
most common inherited hypercoaguable state
VENOUS THROMBOSIS
act protein C (APC) is natural anticoagulant
factor V leiden
hereditary
most common type of APC resistance
increased VENOUS thrombosis
protein C deficiency
hereditary
activated protein C is natural anticoagulant
not enough C=too much clotting (VENOUS)
protein S deficiency
hereditary
protein S is an activator of C
no S=no APC=VENOUS clots
lupus anticoagulant
acquired
increased risk for ARTERIAL CLOTS
lupus anticoag is an PROTHROMBOTIC agent (misnamed anticoag)
malignancy
acquired
most common cause of hypercoaguable state
VENOUS thrombosis
