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Hema > Anemia, Hemoglobinopathies and Thalassemias > Flashcards

Anemia, Hemoglobinopathies and Thalassemias Flashcards

1
Q

Most common anemia among hospitalized patients

A

Anemia of Chronic Inflammation / Anemia of chronic disease

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2
Q

Most commonof the inherited aplastic anemia

A

Fanconi Anemia (FA)

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3
Q

A common site for clinically significant bleeding

A

GIT

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4
Q

May be secondary to trauma, surgery or disease

A

Bleeding

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5
Q

A rare but potentially deadly bone marrow failure syndrome

A

Aplastic anemia

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6
Q

A drug most frequently implicated in acquired aplastic anemia

A

Chloramphenicol

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7
Q

A deficiency of complement regulatory complements

A

PNH aka Marchiafava-Micheli Syndrome

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8
Q

What is the central feature of ACI?

A

Sideropenia (⬇️serum iron) despite abundant iron stores

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9
Q

Master regulatory hormone for systemic iron metabolism and it inhibits ferroportin

A

Hepcidin

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10
Q

Protein that transports iron from tissues to blood

A

Ferroportin

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11
Q

Developed when production of protophorphyrin or the incorporation iron into the protoporphyrin is prevented

A

Sideroblastic anemia

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12
Q

What is the hallmark of sideroblastic anemias?

A

Ringed sideroblasts

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13
Q

The rings in the ring sideroblasts are

A

Mitochondria loaded with iron

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14
Q

Hookworm infx associated with IDA

A

Necator americanus and Ancylostoma duodenale

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15
Q

Definitions:
Koilonychia
Glossitis
Pica
Pagophagia

A

Koilonychia - spooning of fingernails
Glossitis - tongue inflammation
Pica - non-food item cravings, most common- ICE
Pagophagia - craving for ice

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16
Q

Indirect measure of transferrin by measuring its ability to bind iron

A

Total Iron-Binding Capacity (TIBC)

17
Q

1st lab test to be abnormal in IDA or when iron stored begin to decline
A good indicator of iron storage (ferritin) status

A

Serum Ferritin Test

18
Q

Parasite associated with Vit. B12 deficiency

A

Dyphillobothrium latum

19
Q

Generally characterized by antibodies directed against intrinsic factor and parietal cells

A

Pernicious anemia

20
Q

Found in stomach and for the production of HCl and Intrinsic Factor (needed for Vit.B12 absorption)

A

Parietal cells

21
Q

Pathognomic for megaloblastic anemia

A

Hypersegmented neutrophils (≥6 lobes)

22
Q

Root cause of Megaloblastic anemia

A

Impaired DNA synthesis (TTP is not made= Vit.B12 Deficiency)

23
Q

Binds Vit. B12 and releases it to the blood

A

Transcobalamin (previously known as Transcobalamin II)

24
Q

Vitamin B12-transcobalamin complex, the metabolically active form of Vit. B12

A

Holotranscobalamin (holoTC)

25
Q

One of the complications of Hb S. The continuous erection of penis for 4 hrs

A

Priapism

26
Q

Hemoglobinopathies associated with ⬆️oxygen affinity

A

Hb Hiroshima, Hb Rainier, Hb Bethesda

27
Q

Hemoglobinopathies associated with ⬇️oxygen affinity

A

Hb Agenogi, Hb Beth Israel, Hb Yoshizuka

28
Q

Hb S is 80-100% (at only slight decreased oxygen concentrations sickling occurs)

A

Sickle cell anemia

29
Q

Hb S is 20-40% (at @ much lower oxygen concentrations sickling occurs)

A

Sickle cell trait

30
Q

pH requirement for Cellulose Acetate Hemoglobin Electrophoresis

A

Alkaline Buffer (pH 8.4-8.6)

31
Q

Characteristic appearance of Hb SC crystals

A

Washington Monument (found potruding the RBC membrane)

32
Q

Characteristic appearance of Hb CC crystals

A

Bar of Gold (within RBC membrane)

33
Q

Other names of Thalassemias

A

Hereditary Leptocytosis
Mediterranean Anemia

34
Q

Thalassemia belt

A

Extends from Mediterranean East to Middle East, India to Southeast Asia, South to North Africa
Thalassemia minor seems to impart resistance to malaria

Hema (2 decks)

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