Pathology-rbc And Bleeding Disorders Flashcards by Rafa Iglesias

Red cell indeces:

Average content/mass of hemoglobin per red cell

Mean cell hemoglobin

Mean cell volume- ave volume of rbc
Mean cell hemoglobin conc- average hgb in given volume of rbc
Red cell variation of red cell volume- coeeficient variation of rbc volume

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average hgb in given volume of rbc

Mean cell hemoglobin conc

Mean cell hemoglobin- ave volume if red cell expressed in femto litters
Mean cell volume- ave volume of rbc
Red cell variation of red cell volume- coeeficient variation of rbc volume

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Site of hemolysis with:
Anemia
Jaundice
Splenomegally

Extravascular. Main feature is splenomegally bec if sequestration

Intravascular main feature is increased direct bilirubin manifested as jaundice. Other presentations: hemoglobinemia, hemoglubinuria amd hemosiderinuria

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What is the expected peripheral smear in hemolytic anemias

Normocytic normochromic

Primary myelofibrosis also presents with normocytic normochro,ic anemia. It’ fibrotic obliteration of bone marrow spaces

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Types of hemolytic anemia

Hereditary spherocytosis
G6pd deficiency
Sickle cell
Thalasemia
Autoimmune ha
Microangiopatjic ha
Macroangiopathic ha

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Autosomal dominant
Ankyrin and spectrin mutation causing structural changes of rbc
Rbc lacks central pallor
Splenomegally

Hereditary spherocytosis

Spherocytes- rbc lacks pallor

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Aplastic crisis is associated with what virus?

Parvo virus

Hemolytic crisis is assoc with EBV mononucleosis

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Small dark nuclear remnants in rbc in ASPLENIC patients

Howell-jolly bodies seen in heredetary spherocytosis

Other assoc sx: aplastic crisis (parvo virusb19) and hemolytic crisis (EBV)

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X linked recessive
EPISODIC hemolysis triggered usually by infection
Other triggers (antimalarials, sulfonamides. Fava beans)
Protective against what disease?

Malaria

Dx at point: g6pd deficinecy
Reduces nadph to nadph which acts as reducing agents for oxidized rbcs

Recovery phase for hemolysis is heralded by reticulocytosis

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Membrane bound precipitates on denatured globin chains. These can be phagocytized by macrophages leaving a distinct bite in rbc (bite cells)

Heinz bodies seen in g6pd deficiency

Bite cells not appreciated in asplenic patients

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Point mutation in 6th codon affecting the BETA GLOBIN
Replacement of glutamate with valine
Autosomal recessive
Autosplenectomy

Sickle cell anemia

target cells, reticulocytosis, sickle cell, howell jolly bodies (seen also in spherocytosis)

Target cells or codocytes are dehydrated rbc with bulls eye appearance

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Why is sickle cell in infants asymptomatic until 2-4months of age?

Due to high amount of fetal hbg (2alpha, 2gamma)

Adult hbg is 2alpha:2beta

Sickle cell affects only beta globins

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Sickle cell crisis (three tyoes)

Vasoocclusive: dactylitis
Sequestration: splenic enlargement
Aplastic crisis: parvo virus b19 (seen also in spherocytosis)

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Aplastic crisis caused by parvovirus b19 is seen in which condition

Heredetary spherocytosis

Sicle cell anemia

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What is the drug of choice fo sickle cell anemia? And moa?

Hydroxyurea. It increases fetal hemoglobin

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Tyoes of alpha thalasemia

Silent carrier state: deletion of ONE alpha globin chain
Alpha thalasemia trait: TWO globin chains
Hemoglobin h bonding disease- THREe
hydrops fetalis-all four alpha globin

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Tetramers of gamma-globin chains sen in hydrops fetalis thalasemia

Hb barts

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Types of beta thalasemia

Cooley’s anemia or Beta thalasemia major
BT untermediate
BT minor

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Dehydrated rbc with bulls eye appearance is seen in what conditions

Thalasemia
Sickle cell anemia

These are called codocytes ir target cells

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Codocytes
basophilic stripling
Anisocytosis
Poikilocytosis
Microcytic, hypochromic anemia
Chipmunk facies
Crew cut appearance if skull

Thalasemia

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Small peripheral dots in rbc with microcytic hypochromic anemia is seen these conditions:

TAIL: thalasemia, anemia of chronic cause, ida, lead poisoning

The dots are called basophilic stippling

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Treatment of hemochromatosis? If unresponsive, what pharmacologic agent, what moa?

Phlebotomy

Deferoxamine via chelation

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Increcreased compliment mediated rbc lysis c5b-ca
Decreased cd59 expression
!Hemoglobinuria upon waking up
Thrombosis (mcc of fatality)

Paroxysmal nocturnal hemoglobinuria

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Warm agglutinin is mediated by what immuno globulins?

IgG in warm aglutinin autohemolyric anemia
Also seen in methyldopa, sle and cll hemolysis

Another kind is cold mediated autohemolytic anemia, igM mediated seen in Mycoplasma and Mononucleosis
“Cold ice cream is mmm”

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Coobs tests that checks patient's serum for anti rbc surface iG

Indirect coombs test Direct coombs test checks if patients rbs is coated with igG

Damage if rbc thriugh narrowed or obstructed lumina | Usually with cardiac valve prostheiss

Microangiopathic hemolytic anemia

Schistocytes are fragmented rbc, called helmet cells if cut in half Burr cells or echinocytes are rbcs with spikes What condition?

Rbc trauma

Hepatosplenomegally is seen in which conditions?

These are the extravascular site of hemolysis: Heredetary sphenocytosis Sickle cell anemia Thalasemia G6pd is an exemption bec site depends on presence of assaulting factor

Conditions with hemolytic anemia caused by external factors ex drugs, temp

Autoimmune hemolytic anemia Microangiopathic hemolysis Macroangiopathic hemolysis Intrinsic hemolysis- Conditions with hemolytic anemia caused by inherent structural defect

Anemias from diminished erythropoeisis

``` Megaloblastic anemia Pernicious anemia Iron defeciency anemia Anemia of chronic disease Aplastic anemia Pure cell aplasia ```

Macroovacytes (big oval rbcs) Hypersegmented neutrophils Has neurologic symptom Causes are: Impairment if dna synthesis leading to ineeficient vit b12 deficiency Deficiency of nutrients

Megaloblastic anemia sec to vit b12 deficiency Folate acid defficiency has no neurologic symptoms

What parassite is assoc with vit b12 deficiency? Treatment.

Diphyllobotrium latum from raw fish (flatworm) Treat with praziquantel "PRESSiquantel- flats the worm"

``` Megaloblastic Anemia Atrophy of the fundic glands of stomach Intestinalization (with goblet cell formation) Atrophic glossitis (glazed beefy tongue) Autoimmune attacking the parietal cells ```

Pernicious anemia Parietal cells produces intrinsic factor for vit b12 absorption Chief cell- zymigen cells- producing pepsiongen

Test for differentiating megaloblastic anemia with vit b12 deficiency?

Schiling test Stage 1: oral or im vit b12 Stage 2: intrinsic factor Normal 1 normal 2- deficiency Low 1 normal 2- pernicious anemia Low 1 and 2 malabsorption

``` Most common nutritional disorder in the world? Microcytic hypochromic anemia Basophilic stripping Spoon nails (koilonychia) Pica ```

Iron defficiency syndrome Other basophilic stripping and microcytic and hypochromic conditions: "TAIL"

What parasitic infection causes ida? Treatment | .

Hookworms (necator, ancylostoma) -blood suckers | Albendazole

Plummer vinson syndrome. What maliganancy should be ruled out?

Ida Esophageal webs Atrophic glossitis Inc risk of developing colorectal scca in the elderly Ida in the elderly should be checked for colorectal ca.

Most common cause if anemia among hospitalized patients | Increase in il-6 -->hepcidin

Anemia of chronic disease

Inhibits ferriporin function of macrophage reducing transfer of iron frim storage to bone for erythropoeisis

Hepcidin Stimulated by il-6 in anemia of chronic disease

PANCYTOPENIA Hypocellular bone marrow with fat cells Absent splenomegaly Reticulocytopenia

Aplastic anemia Vs. myelodysplastic syndrome--> the bine marrow in mds is hypercellular

Drug known to cause aplastic anemia? Chemical to cause aplastic anemia?

Chloramphenicol | Benzene

Selective hypoplasm of erythroid elements? Thymoma with leukemia (paraneoplastic syndrome) Differentiate with aplAstic anemia

Pure red cell aplasia Anaplastic anemia is PANCYTOPENIA

Causes of Bleeding disorders

Increased vessel fragility Platelet deficiency Platelet dysfunction Derangement of coagulation

NORMAL bleeding parameters Non thrombocytic purpura Seen in scurvy What caused the bleeding disorder?

Increased fragility of vessels

Spontaneous bleeding happen in what platelet level?

20,000/ml Platelet transfusioon threshold

Thrombocytopenia is defined as?

Antiplatelet antibodies Megathrombocytes Spleen congestion What treatment?

Case point: immune thrombocytopenic purpura Most feared complication: ich Tx with glucocorticoids, splenectomy, immunoglobulin or rituximab (anti cd20 antibody)- if there is relapse s/p splenectomy

Thrombotic Thrombocytopenic purpura pentad vs hemolytic uremic syndrome triad?

Fever and neurologic deficits in TTP Common sdx: thrombocytopenia, microangiopathic hemolytic anemia, renal failure Ttp caused by deficiency of ADAMTS13 vWf metalloprotease HUS by shiga like toxin EHEC 157:7 from burger

Increased bleeding time Decreased GP1b leading to defective adhesion Decreased platelet count

Bernard soulier syndrome of platelet dysfunction

Increased bleeding time Decreased GpIIb-IIIa Defective platelet to platelet aggregation NORMAL platelet count

Glanzmann's thrombasthenia

Three Types of coagulation disorders

Von willebramd disease Hemophilia Disseminated intravascular coagulation

Most common inherited bleeding disorder Differentiated from bernard soulier sundrome for having normal platelet count What is the treatment?

Desmopressin and comc factor 8 and vWf Dx at point: von willebrand factor disease. VWf is associated with factor 8. Without it f8 decreases

Most common heredetary disease associated with life-threatening bleeding What is the treatment?

Factor 8 and cryoprecipitate Case: hemophilia A "A, eight"

Also known as christmas factor. Deficient in what coagulation disorder?

Hemophilia B, factor 9 "9 benign (less life threatening bleeding)

``` Consumptive coagulopathy Thrombohemorrhagic disorder (coexistent state) ```

Dissiminated intravascular dissemination

Common factors with ultimate goal to activate f2

10, 2, 5 fibrin

Extrinsic factors

7 With common factors 10 2 5 fibrin

Prothrombine time tests for

Extrinsic + common factor Bleeding time- speed platelet plug is formed. Partial thromboplastic time- intrinsic+common factor

Causes of DIC | STOP Making New Thrombi

``` Sepsis (gram neg) Trauma Obstetric complications (abruptio placenta) Pancreatitis Malignancy Nephrotic syndrome Transfusion ```

Important in diagnosing dic?

D dimer most specific ``` Other ddx: Fibrinogen Platelet count Pt/ptt Fibrin degredAtion products ```

DIC Fibrin thrombi leading to massive adrenal hemorrhage. Renal Complication of meningococcemia and N. meningitidis

Waterhouse-friderichsen syndrome

Dic special situation

Sheehan syndrome: post partum pituitary necrosis Waterhouse friderichsen sdx Toxemiaof pregnancy

Overall most common cause of anemia?

Bleeding Ida is most common anemia of defeciency

Pathology-rbc And Bleeding Disorders Flashcards

(63 cards)