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Heme/Lymph > Buzzwords For Heme/Lymph Final > Flashcards

Buzzwords For Heme/Lymph Final Flashcards

1
Q

This Oxidative state of Fe is required both for heme function and Fe absorption in the gut. (Where does it get absorbed?)

A

Fe2+ - absorbed by DMT-1 in the Duodenum

Fe3+ on Hb is Methemoglobin, which does not carry O2

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2
Q

What is the Committing enzyme of Heme synthesis?

A

Aminolevulinate Synthase (ALAS)

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3
Q

What is the difference between ALAS 1 and ALAS 2?

A

ALAS 1 is found in the liver and is subjected to feedback inhibition by heme (transcription, translation, and translocation into the mitochondria)

ALAS 2 is not inhibited by heme and is found in reticulocytes.

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4
Q

Name 3 things cause increased sickling?

A

1) Increased Deoxygenation
2) Increased HbS
3) Decreased HbF
4) Acidosis
5) Cold Temperature and infections

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5
Q

How do you treat those who are sickling?

A

1) Give suppplemental O2, fluids, and antipyretics

2) Hydroxyurea - stimulate increased HbF production

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6
Q

What are the two types of anemias that produce spherocytes?

What test do you use to differentiate the two?

A

1) Hereditary Spherocytosis
2) Autoimmune Hemolytic Anemia

Use the Coomb’s (Direct Antibody Test) -
Hereditary Spherocytosis (-)
Autoimmune Hemolytic Anemia (+)

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7
Q

What is the Direct Coomb’s test

A

It tests to see if there are anti-bodies DIRECTLY on the surface of RBCs.

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8
Q

Where would you see RBC fragments / Schistocytes

A

Microangiopathic Hemolytic Anemia:

1) TTP - ADAMTS13
2) HUS - E. Coli or Shiga toxin
3) H.EL.LP - High BP during pregnancy
4) Malignant HTN
5) DIC - oozing from injection site

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9
Q

Double Bite Cells

A

G6PD Deficiency

Splenic macrophages removing heinz bodies from RBC.

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10
Q

What is are Heinz Bodies

A

Precipitated Globins with ROS damage

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11
Q

Most important parameter for assessing O2 carrying capacity of the blood

A

Hemoglobin Concentration

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12
Q

What measures the “chromicity” of an RBC?

A

MCHC - Mean Corpuscular Hb Concentration

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13
Q

In acute blood loss, a CBC will often not show anemia. Why is this and when is anemia shown?

A

Blood loss is whole blood, anemia develops when tissue fluid enters the vascular space to restore blood volume.

Reticulocytes will increase after 2-3 days and peak at 7-10 days

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14
Q

Symptoms of Porphoryias with defect EARLY in the biosynthesis pathway

A

Neurological defects - Build up of ALA -> producing ROS

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15
Q

Symptoms of porphoryias with defect in the LATE biosynthesis pathway

A

Photosensitivity

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16
Q

How long after birth are Sickle Cell Symptoms usually seen? Why?

A

10-12 Weeks - It’s the amount of time for a baby’s HbF to go down. HbF is protective against HbS

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17
Q

What is the major cause of death in adults with Sickle Cell Disease?

A

Acute Chest Syndrome

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18
Q

Acute Chest Syndrome

A

Lung clots that arrise from Infection or fat emolism from infarcted marrow; the cycle goes: sluggish flow –> sickling –> vasocclusion* –> more slugish flow

In lungs hypoxia causes constriction to shunt blood away, causing more vasocclusion

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19
Q

Most common cause of death in Sickle cell children?

A

Infection! Children will die because of autoslenectomy, allowing encapsulated bacteria (Strep pneumoniae and Haemophilus influenza).

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20
Q

Alpastic Crisis due to a drop in RBC precursors, is typically due to this?

A

Parvovirus B19

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21
Q

Where diseases do you see Aplastic Crisis?

A

Sickle Cell Anemia, Hereditary Spherocytosis

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22
Q

Percentages of Hb types in Sickle Cell TRAIT

A

HbA 60%, HbS 40% (If you see HbS x > 50% then there will be sickling) unless you look at the kidneys (Renal medulla) which are already hypertonic.

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23
Q

Thalassemia’s complication are directly related to what?

A

Deletions (alpha thalassemias) or Mutations (Beta thalassemias) that cause a decrease or absence of globin units.
Excess normal flobin chains accumulate and cause intracellular RBC cell death (ineffective erythropoiesis) or decreased survival (hemolytic anemia)

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24
Q

What genetic change causes Beta Thalassemia?

A

Mutation (Splicing error) in the B gene on chromosome 11?

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25
Q

What genetic change causes Alpha Thalassemia?

A

Deletion of the alpha gene on chromosome 16?

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26
Q

What is the hall mark lab finding for Beta Thalassemia Minor?

A

HbA2 increases to about 5% (from 2.5%)

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27
Q

What is the chemical marker of hemolysis due to its binding of free hemoglobin

A

Haptoglobin

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28
Q

What does Haptoglobin bind to? What is it cleared by

A

Haptoglobin binds to Hb

Haptoblobin-Hb is cleared by CD 163 - a scavenger receptor on monocytes/macrophages

29
Q

What happens when CD163 binds its ligand? What is its ligand?

A

When CD163 binds Haptoglobin-Hb it causes receptor mediated endocytosis via clatherin coated pits;
Haptoglobin is then degraded and CD163 is recycled back to the membrane

30
Q

Hemolysis causes falsely HIGH or LOW lab values

A

Falsely High

When our of the RBC, Hb absorbs in the measured wavelengths

31
Q

Autosplenectomy is seen in almost all adults with this condition

A

Sickle Cell Disease

32
Q

Excess Bleeding with circumcision tips you off to what genetic bleeding disorder? What pathologies could be seen?

A

Hemophilia A or B (VIII or IX)

You can also see excess bleeding from the cutting of the umbilical cord.

33
Q

What inheritance is Hemophilia A or B

A

X-linked

34
Q

What is the most common anemia overall?

A

Iron Deficiency Anemia

35
Q

What is the most common Hospital patients

A

Anemia of Chronic disease

36
Q

Heme Synthesis enzymes inhibited in lead poisoning

A

ALAD and Ferrochetalase.

Pb displaces Zn in ALAD, causing a build up of ALA - > Neurological defects

37
Q

Why would you want to get genetic testing for Alpha Thalassemia Trait (2 deletions)?

A

Because you want to determine if it Cis or Trans Thalassemia

38
Q

Which Haptoglobin subtype is associated with autoimmunity and disruption of HDL

A

Hp2-2.
Hp2-2 binds ApoA which facilitates the loss of LCAT and decreases antioxidant activity. -> Marker for coronary artery disease

39
Q

Sx seen in pts. with sickle cell trait

A

Microhematuria and decreased ability to concentrate urine.

40
Q

What binds free heme in the blood?

A

Hemopexin. Used when Haptoglobin is overwhelmed. Hb -> metHb + heme. hemopexin takes the free heme off of albumin. Then hemopexin-heme bind to CD91 and into macrophages.

41
Q

Location of GI absorption of anemia causing substances. (Iron, Folate, Vit B12)

A

Iron - Duodenum
Folate - Jejunum
Vit. B12 - Iliuem

I’D /Fight Justin /Beiber Instantly

42
Q

When Fe levels are sufficient, what liver protein inhibits ferroportin? (On enterocytes and macrophages)

A

Hepcidin

Blocks Fe transport into the blood via ferroportin
Suppresses EPO production

43
Q

What is the most sensitive and cost effective test for iron deficiency anemia?

A

Serum Ferritin

Low Ferritin
High RDW
High FEP
Low Serum Iron
Low % Saturation
High TIBC
44
Q

Deletion of 3 alpha globin genes leads to what disease

A

HbH disease - B tetramers, soluble so it will not precipitate in normoblasts but will in peripheral RBC resulting in hemolysis.

45
Q

Infant with Sickle Cell presents with Hb x

A

Splenic Sequestration

46
Q

Why are many patients with Iron defienct or Megaloblastic anemia relatively asymptonmatic despite low Hb?

A

They are asymptomatic due to a physiologic compensation when deficiecy of Fe, Folate, or B12 is over a long period of time.

47
Q

Symptoms of Iron Deficiency Anemia

A

Koilonychia (Spooning of the fingernails)
Pica - Compulsion to eat/chew on non-food (dirt/ice)

Treat Pica with Ferritin Sulfate

48
Q

How long does it take for Folate stores to be depleated?

A

Months

Deficiency is caused by:

1) Poor Diet
2) Increase Demand
3) Drugs - Methotrexate -> inhibits dihydrofolate reductase preventing the reuse of folate
4) Jejunal resection

49
Q

Why is folate supplementation crucial in pregnancy for patients with Folate deficiency?

A

Without you can get neural tub defects/Anencphaly associated with deficient folate

50
Q

What helps you differentiate between Folate and VitB12 deifciency

A

Methymalonic Acid

51
Q

Most common cause of Vitamin B12 deficiency?

A

Pernicious Anemia.
Deficient vitamin B12 secondary to decreased intrinsic factor from gastric parietal cells; MUST have an autoAb against intrinsic factor for diagnosis.

52
Q

How long does it take to develop a VitB12 Deficiency

A

Years. Due to the massive stores in the liver.

53
Q

Treatment for Beta Thalassemia Major

A

Transfusions with Aggressive Iron Chelation
Major = Transfusion dependent (Hb is around 2-3g/dL)
Without Aggressive Iron Chelation you would get endocrine and cardiac disease

54
Q

Vitamin B12 deficiency is associated with what poor diet and what parasite

A

Strict vegan* diet and Diphyllobothrium latum (fish tapeworm).

*Eggs and dairy both contain vit B12 so not vegetarians; also associated with ilieal resection (site of resorption)

55
Q

What three thigs require VitB12 for production

A

1) Tetrahydrofolate - decrease -> Impaired DNA
2) Methionine
3) Succinyl-CoA

56
Q

What is the characteristic intracellular findings and morphology (respectively) associated with G6PD Deficiency?

A

Heinz Bodies and Bite Cells

Heinz Bodies are precipitated Hb globins that had ROS damage. These are removed by splenic macrophages resulting in “Bite Cells”

57
Q

Increased homocysteine in Megaloblastic Anemia increases risk for what complication?

A

Venous and Arterial Thrombosis

58
Q

What genes are most commonly mutated in a patient with hereditary spherocytosis?

A

Anikarin, spectrin, and Band 3

59
Q

Treatment for Hereditary Spherocytosis will result in the presence of what on blood smear?

A

You need to get a SPLENECTOMY!

A Splenectomy will result in Howell-Jolly Bodies and acanthocytes.

60
Q

What two infections are associated with cold agglutinins?

A

Mycoplasma Pneumoniae - anti I

Infectious Mononucleosis - anti i

61
Q

A patient presents with fatigue, decreased propioception on their legs, and spastic paresis. What substrate has built up and where?

A

Methylmalonic acid has build up in the dorsal and lateral tracts of the spinal cord causing demyelination.

62
Q

A Person with pancreatic insufficiency is subjected to what type of anemia

A

Vitamin B12 deficient anemia.
Pancreatic enzymes cleave R-binder, which release Vit. B12 from R-binder and allows it to bind to IF in the small intestine.

63
Q

Why should folate not be used to treat Vitamin B12 deficiency?

A

Folate will cure the anemia, but will NOT treat the neurologic symptoms

64
Q

What is the essential test for diagnosis of Autoimmune hemolytic anemia?

A

Direct Coombs Test

65
Q

What disease is most commonly associated with Warm Autoimmune Hemolytic Anemia?

A

SLE

66
Q

What drugs are most commonly associated with Warm Autoimmune Hemolytic Anemia?

A

Methyldopa & Penicillin/Cephalosporin

Penicillin causes a response while bound to the RBC while methyldopa causes generation of AB against RBCs

67
Q

A patient presents with acrocyanosis, Raynaud’s phenomenon, and anemia, what isotype of AB is elevated in their blood?

A

IgM

Agglutination exasperated with cold exposure. Hemolysis due to COMPLEMENT!

68
Q

In G6PDH Deficiency, protection from oxidative stress is lacking because no _____ is produced so __________ is not made

A

In G6PDH Deficiency, protection from oxidative stress is lacking because no NADPH is produced so Glutathione is not made

Heme/Lymph (18 decks)

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