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
One of the complications of Hb S. The continuous erection of penis for 4 hrs
Priapism
26
Hemoglobinopathies associated with ⬆️oxygen affinity
Hb Hiroshima, Hb Rainier, Hb Bethesda
27
Hemoglobinopathies associated with ⬇️oxygen affinity
Hb Agenogi, Hb Beth Israel, Hb Yoshizuka
28
Hb S is 80-100% (at only slight decreased oxygen concentrations sickling occurs)
Sickle cell anemia
29
Hb S is 20-40% (at @ much lower oxygen concentrations sickling occurs)
Sickle cell trait
30
pH requirement for Cellulose Acetate Hemoglobin Electrophoresis
Alkaline Buffer (pH 8.4-8.6)
31
Characteristic appearance of Hb SC crystals
Washington Monument (found potruding the RBC membrane)
32
Characteristic appearance of Hb CC crystals
Bar of Gold (within RBC membrane)
33
Other names of Thalassemias
Hereditary Leptocytosis Mediterranean Anemia
34
Thalassemia belt
Extends from Mediterranean East to Middle East, India to Southeast Asia, South to North Africa Thalassemia minor seems to impart resistance to malaria