Pathology Flashcards
What happens to the CBC in acute blood loss anemia?
There are proportional decreased in blood count, including WBC and platelets.
What happens to reticulocyte production in acute blood loss? Does it increase or decrease?
Increases
This is the condition where there are premature destruction of RBC’s, ↑ EPO levels, and accumulation of Hb degredation products.
Hemolytic anemia
Is there more unconjugated or conjugated forms of bilirubin in hemolytic anemia?
Unconjugated
since theres an overload of Hb that can’t all be conjugated imemdiately
What happens to the levels of erythroid precursors/normoblasts in the marrow in hemolytic anemia?
Normoblasts ↑
This is the hemolysis of RBC’s within vessels.
Intravascular hemolysis
What is the protein responsible for picking up free Hb?
Haptoglobin
When haptoglobin’s reserves are depletes, what will the rest of the free Hb eventually be oxidized to?
Methemoglobin
So in intravascular hemolysis, what happens to the following values?
Blood Hb
Urine Hb
Urine hemosiderin
Blood haptoglobin
Blood Hb ↑ (hemoglobinemia)
Urine Hb ↑ (hemoglobinuria)
Hemosidinuria
↓ serum haptoglobin
If there is hemolysis in places other than the vessels, what is that condition called?
Extravascular hemolysis
What is the main organ that causes extravascular hemolysis?
Spleen’s sinusoids
Why would the spleen be a little biatch and break down RBC’s? What might be wrong with the RBC’s?
the RBC’s can become less deformable –> get stuck in the spleen sinusoids –> get phagocytoses and broken down
What happens to the gross spleen in extravascular hemolysis diseases?
Splenomegaly
This is an inherited defect int eh RBC cytoskeleton (tethering proteins) which causes weird RBC shapes and extravascular hemolysis?
Hereditary spherocytosis
What are the 3 main proteins affected by hereditary spherocytosis?
Spectrin
Ankyrin
Band 3.1
If theres loss of cytoskeletal proteins in herediatry spherocytosis, what might happen to the the SHAPE of the RBC?
It causes it to be spheres (lol it’s in the name) instead of disc-shaped
If there are sphere-shaped (pac man ghost shaped) RBC’s in hereditary spherocytosis, what happens in the spleen?
They can’t get through the sinusoids –> consumed by splenic marophages –> anemia
What happens to the color of the RBC’s in herediatary spherocytosis?
They lose their central pallor (cuz they’re all big and fat)
What happens to the RDW levels and MCHC levels in hereditary spherocytosis?
RDW ↑
MCHC ↑
What are the clinical manifestations of herediatary spherocytosis?
Splenomegaly
Jaundice with unconjugatted bilirubin (like any other hemolysis)
Biliribin gallstones
What is the test u should do to test for hereditary spherocytosis?
Osmotic fragility test
What is the 1 treatment for hereditary spherocytosis?
Splenectomy
After the splenectomy in hereditary spherocytosis, what might still appear in RBC’s on blood smear?
Howell Jolly bodies
fragments of nuclear material
This is a XR disorder wher ethe HMP shunt or glutathione metabolism are problematic.
G6PD deficiency
What is the main product of the HMP shunt (mainly by the G6PD enzyme), which helps reduce glutathione?
NADPH
What can the reduced glutathione break down so there arent oxidative injuries and hemolysis?
H2O2 (and other free radicals)
What is the form of G6PD deficiency where there is a mildly reduced half-like of G6PD, leading to mild hemolysis with oxidative stress?
African variant
What is the form of G6PD deficiency where there is MARKEDLY reduced half-like of G6PD, leading to marked intravascular hemolysis with oxidative stress?
Mediterranean variant
Regardless of the variant, G6PD deficiency has a protective role against which infection?
Falciparum malaria
think like theyre always a little under stress and the malaria that enters the RBC just gets pimp slapped
The oxidative stress in G6PD deficiency causes the formation of what of RBC inclusions?
Heinz bodies
ketchup in G6PD deficiency
What type of things can cause oxidative stress and are not warrarented in G6PD deficiency?
infections
drugs (primaquine, sulfa drugs, and dapsone)
Fava beans
In G6PD deficiency, when the Heinz bodies are removed from the RBC’s by splenic macrophages, they take a bite outta the RBC, causing the change to which morphology?
Bite cells
What is the presentaton of G6PD deficiency hourse after exposure to oxidative stress?
Hemoglobinuria and back pain
So to screen for G6PD deficiency, what cells do you look for?
Heinz bodies and bite cells
This is a disease where a point mutation in the 6th codon substitutes a Bal for a Glutamate.
Sickle cell
When there are low levels of what do you see polymerization of HbS and thus the sickle shape?
Oxygen
What form of Hb inhibits HbS, sometimes make it hard to Dx in infance?
HbF
What is the inheritance of HbS?
AR
What infections is HbS act as a protective role?
P. falciparum
In sickle cell disease, which system removies RBCs with damaged membranes, leading to anemia, jaundice with unconjugated hyperbilirubinemia, an increased risk for bilirubin gallstones (extravascular hemolysis patterns)?
Reticuloendothelial system (RES)
In sickle cell, if there is intravascular hemolysis, there is dehydration of the RBC cytoplasm, leading to what RBC morphology?
Target cells
In sickle cell disease, what happens to haptoglobin levels as intravascualr hemolysis persists?
↓ haptoglobin levels
they’re all getting bound by the new free Hb
If there is ANY hemolysis, what happens to erythroid precursors?
They undergo hyperplasia to compensate
In sickle cell disease, there is an expansion of hematopoieses into the skull and facial bones, leading to what facial morphology?
“Crewcut” appearance and chipmunk face
In sickle cell, irreversible sickling can lead to this condition, where there is swollen hands and feet due to vaso-occlusive infarcts int he bones, commonly in infants.
Dactylitis
In sickle cell, irreversible sickling reads to a this conition, which is characterized by a shrunked, fibrotic spleen.
Autosplenectomy
lol literally u take out ur own spleen
If there is autosplenectomy in sickle cell, what 3 bugs are u at risk for getting?
Encapsulated organisms, such as…
Strep pneumoniae
H influenza (most common cause of death in children)
Ssalmonella paratyphi osteomyelitis
What bodies are seen on blood smear in sickle cell>
Howell-Jolly bodies
In sickle cell disease, this is the condition where there is vaso-occlusion in the pulmonary microcirculation, leading to chest pain, SOB, lung infiltrates, and often precipitated by PNA.
Acute chest syndrome
Acute chest syndrome is the most common cause of death in what sickle cell age pts?
Adults
And again, what is the most common cause of death in kids with sickle cell?
H. influenza from autosplenectomy
What is the renal manifestations of Sickle Cell disease?
Renal papillary necrosis (hematuria and proteinuria)
remember the “SOAP” acronym for papillary necrosis?
This is the form of sickle cell condition where there is the presenc of 1 mutated and 1 normal.
Sickle Cell TRAIT
just like being heterozygous
True or False: sickle cell trait is generally asymptomatic as there is never any sickling of the cells.
FALSE
There is sickling of the RBC’s with <In which50% except in the renal medulla
In which condition (disease or trait) do you see sickle and target cells on blood smear?
Sickle cell disease
What is the screen for sickle cells which causes cells with ANY amount of HbS to sickle, yielding a + result in both disease and trait?
Metabisulfite screen
Infection with which virus can complicate the already poor production and cause aplastic crisis (low reticulocyte count) in sickle cell disease?
Parvovirus B19
Generally, this is anemia due to decreased synthesis of the globin chains of Hb, which leads to microcytic anemia.
Thalassemia
Which infections are thalassemias good at resisting?
P. falciparum
How many alpha-globin genes do we have on chromosome 16?
4
What is the Sx of a-thalassemia with 1 gene deleted?
There arent any.
What is the Sx of a-thalassemia with 2 genes deleted?
mild anemia with increased RBC count
What is the form of a-thalassemia with 2 genes deleted where there an increased risk of severe thalassemia in the OFFSPRING?
cis deletion
Which demographics are at risk for cis deletions of the a-globin?
Asians
Which demographics are at risk for trans deletions of the a-globin?
African Americans
What is the Sx of a-thalassemia with 3 genes deleted?
Severe anemia
This is the form of Hb when 3 alpha-globin genes are deleted, forming a tetramers of B-chains, which can damage RBC;s.
HbH
What is the Sx of a-thalassemia with 4 genes deleted?
YOU DEAD
hydrops fetalis
This is the form of Hb where there are tetramers of gamma-globin chains
Hb Barts
Instead of deleting the whole gene, where on the DNA is there deletions to cause B-thalassemia?
usually in the PROMOTER region
This is the minor form of B-thalassemia, and is typically asymptomatic, an ↑ RBC count, and u see microcytic hypochromic RBC’s on blood smear.
ß-thalassemia minor (ß/ß+)
What special form of RBC’s do u see on blood smear for ß-thalassemia minor?
(think low cytoplasm)
target cells
For ß-thalassemia minor, what do u see on Hb electrophoresis?
↓ HbA with isolated HbA2
This is the form of ß-thalassemia which is more severe, there is high HbF, and alpha tetramers of aggregate and damage RBCs.
ß-thalassemia major (Bo/Bo)
Do you see extravascular or intravasuclar hemolysis in ß-thalassemia major?
Extravascular
ß-thalassemia leads to the same massive erythroid hyperplasia problems (weird skull, extra medullary hematopoiesis with hepatosplenomegaly, and risk of aplastic crisis with parvovirus B19) as what other extravascular hemolysis disease?
Sickle cell disease
What is the main treatment for ß-thalassemia?
Chronic transfusions
What is the main complication with chronic transfusions in the treatment of ß-thalassemia?
Secondary hemochromatosis
In addition to microcytic, hypochromic RBC’s and target cells, what type of RBC’s do u see in ß-thalassemia, as if they were make in the spleen or liver?
Nucleated RBCs
This is a defect in myeloid stem cells resulting an absent GPI, rendering cell susceptible to destruction by complement.
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Is PNH inherited or acquired?
Acquired
What is the molecule on the surface of normal RBC’s which inhibits C3 convertase, which protects it from complement-mediated damage.
Decay Accelerating Factor (DAF)
AKA CD55
Which molecule secures DAF to the RBC membrane? It also secures CD59 to the membrane as well.
GPI
In PNH, there is a lack of GPI, leading to what problem?
Complement-mediated damage
What happens during the nightime, which causes the nocturnal hemoglobinuria?
Mild respiratory acidosis –> activation of complement –> lysis of RBCs and WBCs and platelets
In PNH, the intravascular hemolysis at night leads to what changes in the blood and urine?
hemoglobinemia
Hemoglobinura
Hemosidinuria (seen days after hemolysis)
What is the test that is used to screen for PNH?
Sucrose test
What is the confirmatory test for PNH?
Acidified urine test or flow cytometry to detect the lack of CD55 (DAF) on blood cells.
What is the main cause of death in PNH?
Thombosis of hepatic, protal, or cerebral veins
What are the 2 main complications to PNH?
Iron deficiency anemia
Acute myeloid leukemia (AML)
This is the antibody-mediated (IgG or IgM) destruction of RBCs.
Immune hemolytic anemia (IHA)
Which IHA involved EXTRAvascular hemolysis (IgM or IgG)?
IgG-mediated
In IHA, when does the IgG’s bind to the RBC, which is then consumed by splenic macropahges, leading to spherocytes?
In warm temperature (warm agglutinin, like in the central body)
What is the autoimmune disorder associated with IgG IHA?
SLE
PCN and cephalosporins can cause IgG IHA because of what mechanism?
Drug attaches to RBC membrane –> Ab attaches to drug –> extravascular hemolysis
The drugs (like a-methyldopa) that can cause IgG IHA can leave a resdual effect, where you might start producing what molecules, leading to extravascular hemolysis?
Autoantibodies
What is the treatment for IgG IHA?
Cessation of the offending drug, steroids, IVIG, and if necessary, splenectomy
What is the IHA conditon that involved intravascular hemolysis?
IgM-mediated
Under what condition does IgM bind RBCs and fix complement?
Relatively cold temperatures
cold agglutinin, like in the extremities
What 2 infections is associated with IgM IHA?
Mycoplasma pneumoniae and infectious mononucleosis
This is the test that is used to Dx IHA.
Coombs test
This is the form of Coombs test where Anti-IgG is added to pts RBCs, and see if agglutination occurs (they do if RBC’s are already coated with Ab).
Direct coombs test
What is the procedure for the INdirect coombs test?
It sees in Ab’s are in the SERUM…
anti-IgG and test RBCs are mixed with the pts serum, and agglutination occurs if the serum antibodies are present.
This is the condition where there is microangiopathic hemolytic anemia from O157:H7 bacteria.
HUS
What is the O157:H7 toxin that damages endothelial cells, leading to platelet microthombi?
Verotoxin (pathoma version)
Shiga or Shiga-like toxin (Moscatello version)
In normal B12 metabolism, what is bound to B12 as it enters the mouth and protects it until it reaches the duodenum?
R-binder
Once the B12 reaches the duodenum, it gets complexed to Intrinsic Factor, which come from what GI cells?
Gastric parietal cells
Where in the bowel is the B12-IF complex absorbed?
Terminal ileum
After how long do u need to develop B12 deficiency? Why?
YEARS
the liver stores a lot of it, so u see this in strict vegans and stuff
This is the most common anemia to cause B12 deficiency, where there is autoimmune destruction of parietal cells, leading to the absence of IF.
Pernicious anemia
What can cause damage to the terminal ileum, leading to B12 deficiency from a lack of uptake from the GI?
Chron disease
Diphyllobothrium latum tapeworm
Both B12 and folate deficiencies lead to what morphological change in RBCs?
Macrocytic RBCs
What happens to the neutrophils in B12 deficiency?
Theyre hypersegmented
What happens to the tongue in B12 deficiency>
Glossitis
What happens in the spinal cord in B12 deficiency?
Subacute combined degeneration
(B12 is required for the conversion of methylmalonic acid –> succinyl CoA for FA metabolism, so it impairs spinal cord meylinization)
For blood labs, what happens to vitamin B12, serum homocysteine, and methylmalonic acid levels in B12 deficiency?
B12 ↓
↑ serum homocysteine (↑ risk for thrombosis)
↑ methylmalonic acid (unlike folate deficiency)
Folate and B12 are necesary for the synthesis of what in the cell?
DNA
In order to be transferred from methyl THF to folate, what molecule must pick up the methyl group?
B12
The Methyl-B12 molecule then transfers the methyl to which other molecule to produce methionine?
Homocyteine
Where in the gut is folate absorbed?
Jejunum
How long must it take to develop a folate deficiency?
Months
In what type of pts do you see a folate deficiency from poor diet?
Alcoholics and the elderly
In what type of pts do you see a folate deficiency from increased demand?
Pregnancy, cancer, ane hemolytic anemia
basically any time you are using a lot of folate to make new DNA
In what type of pts do you see a folate deficiency from folate antagonists?
Pts taking metrhotrexate, which inhbiits DHF reductase)
What happens to the tongue in folate deficiency, like in B12 deficiency?
Glossitis
What is the RBC morphology in folate deficiency, like in B12 deficiency?
Macrocytic RBCs
What is the neutrophil morphology in folate deficiency, like in B12 deficiency?
Hypersegmented neutrophils (> 5 lobes)
What are the serum levels of folate, homocysteine, and methylmalonic acid in folate deficiency?
Folate ↓
↑ homocyteine
NORMAL methylmalonic acid (in B12 deficiency it’s ↑)
This is the type of anemia from ↓ iron levels, and is the most common type of anemia.
Iron deficiency anemia
What is the RBC morphology in Iron deficiency anemia?
hypochromic microcytic
Where in the gut is iron absorbed?
Duodenum
What is the transporter on enterocytes that absorbed Fe into the cell?
DMT1
What is the molecule that transports iron across the cell membrane into the blood?
Ferroportin
What is the molecule that transports Fe in the blood and delivers it to liver and bone marrow macrophages for storage?
Transferrin
What is the stored intracellular iron bound to, which prevents Fe from forming free radicals via the Fenton rxn?
Ferritin
This is the measure of transferrin molecules in the blood,
TIBC
total iron-binding capacity
True or False: the serum ferritin accurately reflexts iron stores in macrophages and the liver.
True
Why do infants get Iron deficiency anemia?
Breast feeding (human milk is low in iron)
Why do kids get Iron deficiency anemia?
poor diet
What is the main cause in male and female adults in Iron deficiency anemia?
Males- peptic ulcer disease
Females- Menorrhagia or pregnancy
What is the main cause of Iron deficiency anemia in the elderly in the Western world?
Colon polyps/carcinoma
What is the main cause of Iron deficiency anemia in the elderly in the developing world?
Ancylostoma duodenale and Nieator ameicanus
What happens to the ferritin and TIBC levels when storage iron is depleted in Iron deficiency anemia?
↓ ferritin
↑ TIBC (liver makes more transferrin to seek out more Fe)
What is the intial stage of Iron deficiency anemia, characterized by bone marrow making fewer, but normal sized RBCs?
Normocytic anemia
Normocytic anemia progresses to what RBC morphology in Iron deficiency anemia?
Microcytic hypochromic anemia
This is the condition of Iron deficiency anemia where there is spoon-shaped nails.
Koilonychias
This is the condition of Iron deficiency anemia where u eat random crap like dirt and hair.
Pica
Why is the Free erythrocyte protoporphyrin (FEP) levels ↑ in Iron deficiency anemia?
Cuz when heme breaks down to Fe and protoporphyrin, since Fe is ↓ and protoporphyrin in normal, it gets free in the RBC
What is the treatment for Iron deficiency anemia?
Supplemental iron (ferrous sulfate) and the underlying cause
This is a manifestation of Iron deficiency anemia where there is esophageal webs and atrorophic glossitis, and presents as anemia, dysphagia, and a beefy-red tongue.
Plummer-Vinson syndrome
What is the molecule that inhibits Ferriportin, which normally allows Ferrous ions out of the cell?
Hepcidin
This is the anemia assocaited with chronic inflammation (endocarditis or autoimmune conditions, and is the most common type of anemia in HOSPITALIZED pts.
Anemia of chronic disease
What is the important molecule produced in Anemia of chronic disease, which sequesters iron storage sites?
Hepcidin
What are the 2 ways hepcidin sequesters iron storage?
- limiting Fe transfer from macrophages to erythroid precursors
- supressing EPO production
What happesn to the ferritin, TIBC, serum iron, and FEP in Anemia of chronic disease?
↑ ferritin
↓ TIBC
↓ serum iron
↑ FEP
In Anemia of chronic disease, what agent is used in the treatment, especially in those with cancer?
Exogenous EPO
In Anemia of chronic disease, what is the role of hiding away all the Fe? What is it trying to prevent from accessing the iron?
Hiding away all the iron prevents the bacteria from accessing iron, which is necessary for their survival
(but it turn it leads to anemia cuz it takes the Fe from your own cells to use)
This is the condition where there is reduced #’s of RBCs, WBCs, and platelets.
Pancytopenia
This is the type of anemia where chemicals, viral infections, radiation, or drugs cause marrow suppression, leading to pancytopenia.
Aplastic anemia
“a-“ = without, “-plastic” = formation –> “without formation” anemia
IF you take a bone marrow biopsy in aplastic anemia, what would the marrow look like?
Empty and fatty
What is the treatment for aplastic anemia?
cessation of caustive drugs and supportive care with marrow-stimualting factors (EPO, GM-CSE, and G-CSE)
What is the last resort treatment for aplastic anemia?
Bone marrow transplant
This is the condition of a primary marrow disorder wehre erythroid progenitors are suppressed.
Pure red cell aplasia.
What viral infection can cause pure red cell aplasia?
Parvovirus B19
In what type of pts does an aplastic crisis occur with Parvovirus B19 infection?
Moderate-severe hemolytic anemias
In addition to parvoviral infections, what causes pure red cell aplasia?
Thymoma + large granular lymphocytic leukemia
Drugs
Autoimmune disorders
This is the condition where a pathological process (like metastatic cancer) replaces bone marrow, causing impairment of hematopoiesis, resulting in pancytopenia.
Myelophthisic process
This is an abnormally high red cell count, usually with a corresponding increase in Hb levels.
Polycythemia
This is the form of polycythemia from dehydration, vomiting, diarrhea, or excessive diuretic use.
Reactive polycythemia
What is the main cause of the primary form of polycythemia (low EPO)?
Polycythemia vera
What is the cause of secondary polycythemia from compensatory mechanisms?
Lung disease
High altitude living
Cyanotic heart disease
What is the cause of secondary polycythemia from paraneoplastic mechanisms?
EPO-secreting tumor
What might the change in Hb itself to cause secondary polycythemia?
Hb mutants with high O2 affinity
What are the 2 things that can cause secondary polycythemia where there is stabilization of HIF-1a?
Chauvash polycythemia
Prolyl hydroxylase mutations
Petechiae and purpuric hemorrhages can form in which infections?
Meningiococcemia
Other forms of septicemia
Infective endocarditis
Several rickettsioses
What is the condition where there is deposition of drug-induced immune complexes in the vessel wall, leading to cutaneous petechiae and purpura?
Hypersensitivity (leukocytoclastic) vasculitis
What 2 disorders can cause microvascular bleeding due to defects in collagen?
Scurvy and Ehlers-Danlos
This is a condition form systemic hypersensitivity of unknown cause, and is characterized by purpuric rask, colicky abd pain, polyarthralgia, and acute glomerulonephritis.
Henoch-schonlein purpura
This is an AD disease where dilated tortuous blood vessels with thin walls can bleed easily, and is common under mucous membranes of the nose, tongue, motuh, eyes, and GI tract.
Hereditary hemorrhagic telangiectasia
Weber-Osler-rendu
Why can perivascular amyloidosis cause mucocutaneous purpura?
Amyloids weaken blood vessel walls and causes bleeding
Under what platelet level do u determine thrombocytopenia?
< 100,000 platelets/uL
What 2 general classes can thrombocytopenias be classifies?
Qualitative (↓ quality) and quanitative (↓ #)
What are the 2 main classical Sx of thrombocytopenia?
- Mucosal bleeding
2. Skin bleeds (petichiae, purpura, ecchymosis, easy bruising)
This is the form of thrombocytopenia where allantibodies are produced in response when platelets are transfused or cress the placenta from fetus into pregnant mother.
Neonatal thrombocytopenia
This is the form of thromboytopenia where massive transfusion can cause a dilutional thrombocytopenia.
Transfusion thrombocytopenia
This is the QUANTITATIVE thrombocytopenia where there is autoimmune production of IgG against the platelets Ag’s (GpIIb/IIIa).
Idiopathic Thrombocytopenic Purpura (ITP)
True or False: ITP is the most common cause of thrombocytopenia in children and adults.
True!
Which cells in the spleen make the autoantibodies for ITP?
Plasma cells
Which cells then consumes the antibody bound platelets in ITP?
Splenic macrophages
Ab’s are made in the spleen and consumed in the spleen
In which ages do u see the acute form of ITP weeks after viral infection or immunzation, and is typically self-limited?
Kids
Who is at risk for the chronic form of ITP?
Women of child bearing age
Though chronic ITP can be idiopathic, what is the main secondary condition that can cause it?
SLE
Since the antiplatelet IgG can cross the placenta in chronic ITP, what might occur in the newborn?
Short-lived thrombocytopenia
What happens to the serum platelet levels, PT/PTT, and megakeryocytes on bone marrow biopsy for ITP?
↓ platelets (usually < 50k/ul)
NORMAL PT/PTT (doesnt touch coag cascade)
↑ megakaryocytes (compensatory hyperplasia)
Kids respond well with what treatment for ITP?
Corticosteroids
What is the treatment for ITP that involves giving splenic macrophages something else to eat other than the platelets?
IVIG
Why is a splenectomy killin 2 birds for the treatment of ITP?
You eliminate the source of Ab and the site of platelet destruction
TTP and HUS are of what class of quantitative thrombocytopenias under what anemia?
Microangiopathic hemolytic anemia
What happens in microangiopathic hemolytic anemia in the small vessels to cause hemolytic anemia?
Formation of platelet microthrombi –> partial lumen occlusion –> sharing of RBCs –> schistocyte formation –> hemolytic anemia
What is the mrophology of the RBC’s in microangiopathic hemolytic anemias?
Shistocytes
pac man ghosts
This is the form of microangiopathic hemolytic anemia where there is microthrombi formation from ADAMTS13 enzyme loss.
TTP
What is the normal role of ADAMTS13 enzyme?
breaks down vWF multimers
In adult females, what is the cause of the loss of ADAMTS13 seen in TTP?
auto-Ab
What is the pentad of Sx in TTP?
Fever Thrombocytopenia Microangipathic hemolytic anemia ****NEURO defects**** Renal failure
HUS has basically the same Sx as TTP except what is enchanced?
RENAL FAILURE
hemolytic UREMIC syndrome
Bernard-soulier, Glanzmann thromasthenia, ASA, and uremia are all what types of thrombocytopenia?
Qualitative disorders
↓ quality of the platelets
What is the deficiency in Bernard-Soulier syndrome, causing problems with platelet adhesion?
GpIb deficiency
What shows up on blood smears for Bernard Soulier syndrome?
Mild thrombocytopenia with enlarged platelets (Big Suckers)
This is the a qualitative thropmbocytopena where there is a deficiency in GpIIb/IIIa, causing problems with platelet aggregation.
Glanzmann thrombasthenia
What product of COX is lost if u give ASA, leading to impairment of platelet aggregation?
TXA2
What problems may cause uremia, which disrupts platelet fxn and leads to both platelet adhesion AND aggregation problems?
Liver or renal problems
How many variants are there for vW disease?
> 20
What is the most common type of vW disease, where there is mild-mod vWF deficiency?
type 1 (AD inherited)
Which type of vW disease shows ectremely low levels of vWF and severe manifestations, and is from frameshift mutations or deletions?
type 3 vW disease
Which test is abnormal in vWF diease (specific for this disease)?
Ristocetin test
What is the treatment of choice for vW disease?
Desmopressin
Which type of vW disease shows qualitative defect in vWF (poor assembly), and has mild-mod bleeding?
type 2A
Which factor is mutated in hemophilia A?
Factor VIII
Which coag pathway is affected by hemophilia A?
Intrinsic pathway
What is the inheritence of hemophilia A?
XR
Where is the bleeding in hemophilia A?
Deep tissue, joint , and postsurgical bleeding
So where is the bleeding in primary hemostasis disorders vs secondary?
Primary- in the mucosa and skin
Secondary- in deep tissues
What happens to the PTT, PT, platelet count,annd bleeding time in hemophilia A?
↑ PTT
everything else normal
What is the treatment for Hemophilia A?
recombinant FVIII
Which coag factor is mutated in hemophilia B?
IX
This is a pathologic activation of the coag cascarde, where there is widespread microthrombi causing ischemia and infarction.
DIC
Which 5 things can cause DIC?
Obstetric complication (amnitotic tissue has thromboplastin) Sepsis Adenocarcinoma (mucus) APL Rattlenake --[][][][][][][][][]D~
(that’s a snake not a ween)
What happens to the platelet count, PT/PTT, and fibrinogen levels in DIC?
↓ platelets
↑ PT/PTT
↓ fibrinogen (cuz u use it all)
What is the specific thing u can screen for in DIC whichi is the product of lysing cross linked fibrin, which tells u the entire coag cascade is activated so much u activate teh splitting of the cross-linked fibrin?
Fibrin split products (D-dimers)
What is teh treatment for DIC?
to the cause, and transfuse and cryoprecipitate if needed
What type of anemia is a manifestation of DIC?
Microangiopathic hemolytic anemia (like in TTP and HUS)
This is a reduction in the # of neutrophils int eh blood.
Neutropenia
This is a clinically significant reduction in neutrophils in the blood, and has a serious consequence of makign pt susceptible to bacterial and findal infections.
Agranulocytosis
What is the most common cause of agranulocytosis?
Drug toxicity
(aminopyrine, chloramphenicol, sulfonamides, chlorpromazine, thiouracil, and phenylbutazone
This the form of leukocytosis which is caused by acute bacterial infections and sterile inflammation.
Neutrophilic leukocytosis
This the form of leukocytosis which is caused by allergic disorders.
Eosinophilic leukocytosis
This the form of leukocytosis which is caused by myeloproliferative disease and is rare
Basophilic leukocytosis
This the form of leukocytosis which is caused by chronic infections, bacterial endocarditis, rickettsiosis, malaria, and collagen vascular diseases and IBD.
Monocytosis
This the form of leukocytosis which accompanies monocytosis in many disorders assocaited with chronic immunological stimulation, viral infections, and Bordetella pertussis infection.
Lymphocytosis
This is a conditon where there is acute lympahdenitis in the cervical region likely due to microbial drainage from infections of teeth/tonsils.
Acute non-specific lymphadenitis
What is the main cause of congestive splenomegaly?
Liver cirrhosis
Where might there be thrombosis or pylephlebitis to cause congestive splenectomy?
Portal v.
What can cause splenic rupture?
BLUNT TRAUMA
What are the 4 predisposing factors for splenic rupture?
Mono
Malaria
typhoid fever
Lymphoid neoplasms
Why are enlarged spleen UNlikely to rupture?
because of the toughening effect of extensive reactive fibrosis
