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Heme and Lymph Final > DSA: RBC Disorders > Flashcards

DSA: RBC Disorders Flashcards

1
Q

essentials of diagnosis (EOD) for iron deficiency anemia?

A
  • if serrum ferritin is < 12 ng or less than 30
  • caused by bleeding unless proved otherwise*
  • responds to iron therapy
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2
Q

Tx of iron deficiency anemia

A

identification of the cause

-especially if it’s occult blood loss

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3
Q

EOD for anemia of chronic disease

A
  • mild or moderate normocytic or microcytic anemia
  • normal or increased ferritin and normal or reduced transferrin
  • underlying chronic disease
  • there’s usually inflammation going on… hepcidin gets released… no iron comes in
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4
Q

what helps us distinguish anemia of chronic disease from iron deficiency anemia?

A

look at the serum ferritin

  • if that is high, then we have plenty of iron
  • if low, iron deficiency
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5
Q

is treatment OF THE ANEMIA necessary for management of anemia of chronic disease?

A

no

-just address the condition causing the anemia of chronic disease

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6
Q

EOD for thalassemias

A
  • microcytosis disproportionate to the degre of anemia
    • family hx
  • lifelong personal history of microcytic anemia
  • normal or elevated RBC count
  • abnormal rbc morphology with microcytes, hypochromia, acanthocytes, and target cells
  • in beta-thalassemia, elevated levels of hbg A2 or F
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7
Q

what is thalassemia?

A

reduction in synth of globin chains

  • hemoglobin= heme + globin
  • Heme= Fe + porphoryn
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8
Q

what is MCH?

A

mean corpuscular hemoglobin

-it’s low in thalassemias

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9
Q

what region of the world are alpha thalassemias seen in a lot?

A

SE asia and China

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10
Q

Who does Beta thalassemia affect the most

A

mediterranean ppl

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11
Q

Alpha thalassemia trait

A

-mild anemias
-low MCV
-normal iron and reticulocyte count
-

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12
Q

Hemoglobin H disease

A
  • more marked anemia
  • low MCV
  • microcytosis
  • reticulocytes elevated
  • electrophoresis will show a fast migrating HbH
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13
Q

Beta thalassemia minor

A
  • in contrast to alpha thalassemia, there is basophilic stippling
  • elevation in HbA2
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14
Q

Beta thalassemia intermedia

A
  • elevated reticulocyte count
  • basophilic stippling
  • there can be lots of HbF
  • similar to minor
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15
Q

Beta thalassemia major

A
  • sever anemia
  • poikilocytosis and whatnot, basophilic stippling
  • nucleated RBC’s
  • very little HbA
  • predominant is HbF
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16
Q

What should we give people that have to get a lot of transfusions?

A

iron chelators

-or else everyone else’s iron will get into them

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17
Q

What is the tx of choice for B thalassemia major and the only available cure?

A

stem cell transplantation

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18
Q

EOD for Vit B12 deficiency

A
  • macrocytic anemia
  • megaloblastic blood smear: macro-ovalocytes and hypersegmented neutrophils
  • low serum vitamin B12 level
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19
Q

What makes Vit B12 deficiency a little unique from folate deficieny?

A

there are neuro symptoms

-because methylmalonic acid builds up

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20
Q

if we find not a lot of B12 in the blood, what confirmatory test can we do to make sure that it’s B12 deficiency?

A

methylmalonic acid or homocysteine

-if high, we got b12 deficiency

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21
Q

How do we tell iron deficiency apart from thalassemia minor?

A
  • thalassemia pts have a lower MCV and a more abnormal peripheral smear and usually reticulocytosis
  • look at the freakin iron studies
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22
Q

how is the diagnosis of alpha thalassemia madE?

A

by exclusion

-there is no change in proportion of the normal adult hemoglobin species

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23
Q

how is B12 decifiency treated?

A

with parenteral therapy

-means inject it into them

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24
Q

EOD for folic acid deficiency

A
  • macrocytic anemia
  • megaloblastic blood spmear
  • reduced folic acid levels in rbc’s or serum
  • NORMAL B12 LEVELS
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25
Q

What should always be measured if folic acid deficiency is suspected?

A

vitamin B12

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26
Q

What kind of patients will get folic acid deficiency?

A

alcoholics!!!

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27
Q

how is folic acid deficiency treated?

A

with daily oral folic acid

-reticulocytosis will happen and everything will be back to normal

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28
Q

EOD for Paroxysmal Nocturnal Hemoglobinuria

A
  • episodic hemoglobinuria
  • thrombosis is common
  • suspect in confusing cases of hemolytic anemia or pancytopenia
  • flow cytometry demonstrates deficiencies of CD55 and CD59
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29
Q

What happens in PNH?

A
  • RBCs get destroyed by complement
  • the Hb binds up NO… vasospasm
  • reddish brown urine in the morning
  • drop in blood pH while sleeping facilitates hemolysis
30
Q

lab findings in PNH?

A

-high serum LD

31
Q

tx of PNH

A
  • no intervention
  • if it’s that bad and you find out they have aplastic anemia, just do stem cell transplantation
  • ecluzimab binds C5 and helps
32
Q

EOD for G6PD deficiency

A
  • X linked recessive disorder seen commonly in american Black men
  • Episodic hemolysis in response to oxidant drugs or infection
  • bite cells and blister cells on the peripheral blood smear
  • reduced levels of G6PD between hemolytic episodes
33
Q

What does G6PD normally do?

A

it’s an antioxidant… so without it our RBC get destroyed by oxidants…

34
Q

is G6PD self limited?

A

yeah

-unless you’re mediterranean

35
Q

What does the peripheral blood smear look like with G6PD defiency?

A

bite cells

-HEINZ BODIES!= denatured hemoglobin

36
Q

EOD for sickle cell anemia

A
  • recurrent pain episodes
    • family hx and lifelong hx of hemolytic anemia
  • irreversibly sickled cells on peripheral blood smear
  • Hemoglobin S is the major hemoglobin seen on electrophoresis
37
Q

which kind of hemoglobin retards the sickling process?

A

HbF

38
Q

What do sickled cells lead to?

A

hemolysis

  • ATP to ADP
  • NK cells go there and pulmonary inflammation happens
  • free hemoglobin takes up NO… pulmonary htn
39
Q

tx of sickle cell anemia

A

allogeneic hematopoietic stem cell transplantation is performed before the onset of significant end-organ damage… 80% success

  • hydroxyurea
  • supportive care is the mainstay of tx 1
40
Q

EOD for autoimmune hemolytic anemia

A
  • acquired hemolytic anemia caused by IgG autoantibody
  • spherocytes and reticulocytosis on peripheral blood smear
    • antiglobulin (Coombs) test
41
Q

is there intra or extra vascular hemolysis in AI hemolytic anemia?

A

extravascular

-RBC’s get coated with IgG, eaten up by Macs… spleen and liver enlarged

42
Q

tx of AI hemolytic anemia?

A
  • prednisone
  • give incompatible blood? might as well
  • Rituximab (CD20 ab…
  • splenectomy if prednisone doesn’t work
43
Q

EOD for cold agglutinin disease

A
  • increased reticulocytes on peripheral blood smear
  • antiglobulin (Coombs) test positive only for COMPLEMENT
  • positive cold agglutinin titer
44
Q

clinical findings for cold agglutinin disease

A

-mottled or numb fingers
-episodic hemoglobinuria in response to exposure to cold
-reticulocytosis
-

45
Q

tx of cold agglutinin disease

A
  • avoid exposure to cold!
  • Rituximab is tx of choice
  • RBC’s should be transfused through an in-line blood warmer
46
Q

EOD for aplastic anemia

A
  • pancytopenia
  • no abnormal hematopoietic cells seen in blood or bone marrow
  • hypocellular bone marrow
  • reticulocytopenia will be there as well
47
Q

What should definitely NOT be present in aplastic anemia?

A

hepatosplenomegaly

-it makes sense b/c there’s no freakin cells

48
Q

what other things could get confused with aplastic anemia?

A
  • hypocellular forms of myelodysplasia or acute leukemia

- look at the marrow, above has mutations and blasts and stuff

49
Q

tx of aplastic anemia

A
  • supportive care with EPO or filgrastim
  • bone marrow transplantation if severe (neturophils less than 500, platelets less than 20,000, retics less than 1%, and bone marrow cellularity less than 20%)
  • immunosuppression
50
Q

what do we need to know about parvovirus?

A
  • it gets erythrocyte precursor cells
  • in IC ppl, pure RBC aplasia
  • in adults, molecular mimicry to human cytokeratin and TF’s engaged in Hematopoiesis
  • in preggo: hydrops fetalis!
  • look for IgM antibodies
  • RT-PCR is the optimal test
  • in IC ppl, give them IVIG
51
Q

Acute intermittent porphyria EOD

A
  • unexplained abdominal crisis, generally in young women
  • acute peripheral or central nervous system dysfunction; recurrent psychiatric illnesses
  • hyponatremia
  • porphobilinogen in the urine during an attack
52
Q

Where is the mutation in AIP?

A

HMBS

-auto dominant

53
Q

What is AIP caused by?

A

partial deficiency of hydroxymethylbilane synthase activity

-leads to increased excretion of aminolevulinic acid and porphobilinogen in the urine

54
Q

What happens with the Urine in AIP people?

A

it’s normal when freshly voided but turns dark upon standing in light and air

55
Q

tx of AIP?

A

-high carbohydrate diet

56
Q

Porphyria Cutanea Tarda EOD

A
  • noninflammatory blisters on sun-exposed sites, especially the dorsal surfaces of the hands
  • hypertrichosis, skin fragility
  • associated liver disease
  • elevated urine porphyrins
57
Q

What does porhpyria cutanea tarda look like?

A
  • bilstering and fragility of the skin of the dorsal surfaces of the hands
  • facial hypertrichosis and hyperpigmentation are common
58
Q

What else can happen with the skin lesions of this?

A
  • can be from dialysis or certain meds
  • then it’s called, pseudoporphyria
  • the biopsy looks the same as porphyria cutanea tarda, but the urine porphyrins are normal
59
Q

Polycythemial Vera EOD

A
  • JAK2 (V617F) Mutation
  • increased RBC mass
  • splenomegaly
  • normal arterial oxygen saturation
  • usually elevated WBC count and platelet count
60
Q

what cell lines does PCV overproduce

A
  • All three hematopoietic stem cell lines

- but most prominently RBC’s

61
Q

What is Erythroid production independent of in PCV?

A

erythropoietin

-so the serum EPO will be low

62
Q

What do we need to distringuish PCV from?

A

increased RBC count from costricted plasma volume

-spurious erythrocytosis

63
Q

What symptoms will PCV present with?

A
  • headache
  • dizziness
  • tinnitus
  • blurred vision*
  • fatigue
  • pruritis after warm bath
64
Q

What is the hallmark lab value for PCV?

A

hematocrit above 54% in males or 51% in females

65
Q

What do we confirm a diagnosis of PCV with?

A

JAK2 mutation

66
Q

What is secreted by WBC’s that leads to increased Vitamin B12 levels in PCV?

A

transcobalamin III

67
Q

What does marked elevation of WBC’s make us think of?

A

CML

68
Q

What does marked elevation of platelets make us think of ?

A

ET

69
Q

What is there’s abnormal RBC morphology and nucleated RBS in the peripheral blood?

A

Myelofibrosis

70
Q

What is the tx of choice for PCV?

A

phlebotomy

  • if problematic, hydroxyurea
  • if resistant to hydroxyurea, give ruxolitinib
71
Q

What is the major cause of morbidity and morality in PCV?

A

arterial thrombosis

72
Q

Over time, what can PCV convert to?

A

myelofibrosis or CML

Heme and Lymph Final (14 decks)

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