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LHT2 > Disorders of RBCs DSA > Flashcards

Disorders of RBCs DSA Flashcards

1
Q

iron def anemia- essentials of dx

A
  • serum ferritin < 12 or < 30 if also anemic
  • caused by bleeding unless proved otherwise
  • responds to iron therapy
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2
Q

iron def anemia- signs and sx’s

A
  • anemia
  • smooth tongue, brittle nails, spooning of nails, cheilosis
  • esophageal webs (plummer-vinson syndrome)
  • pica
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3
Q

iron def anemia- lab

A
  • anemia with MCV (initially)
  • anemia with low MCV
  • ferritin < 12- indicates depletion of iron stores
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4
Q

other causes of microcytic anemia

A
  • chronic dz
  • thalassemia
  • lead poisoning
  • X-linked sideroblastic anemia
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5
Q

Anemia of chronic dz- essentials of dx

A
  • mild/moderate normocytic or microcytic anemia
  • normal or inc ferritin and normal or reduced transferrin
  • underlying chronic dz
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6
Q

Anemia of chronic dz- types

A
  • anemia of infl- hepcidin inc- iron-restricted erythropoiesis
  • anemia of organ failure- erythropoietin is reduced
  • anemia of the elderly- resistance to RBC prod in response to erythropoietin, dec in erythropoietin prod (dec nephron mass), chronic infl cytokines cause deg in erythropoietin
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7
Q

Anemia of chronic dz- tx

A
  • tx the cause

- if severe anemia (Hg < 10)- RBC transfusions or parenteral recombinant erythropoietin

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8
Q

Thalassemias- essentials of dx

A
  • microcytosis
  • FH
  • lifelong personal hx of microcytic anemia
  • normal or elevated RBC count
  • abnormal RBC morphology with microcytes, hypochromia, acanthocytes, target cells
  • B-thalassemia- elevated levels of hemoglobin A2 or F
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9
Q

Thalassemias- described as

A
  • trait- lab features w/o clinical impact
  • intermedia- occasional RDC transfusion requirement or other clinical impact
  • major- life-threatening, transfusion-dep
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10
Q

Alpha-Thalassemia syndromes

A
  • due to gene deletions
  • asia, china
  • silent carrier- 3 alpha genes
  • Thalassemia minor (trait)- 2 alpha genes
  • Hemoglobin H dz- 1 alpha gene
  • Hydrops fetalis- 0 alpha genes
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11
Q

B-Thalassemia syndromes

A
  • due to point mutations
  • mediterranean pts
  • T minor- Bo, B+ (elevated HbA2)
  • T intermedia- B+ (mild)
  • T major- Bo
  • T major- B+
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12
Q

B-Thalassemia major- sx

A
  • at 6-9 months- anemia requiring transfusions
  • stunted growth, bony deformities
  • transfusional iron overload- HF, cardiac arrhythmias, cirrhosis
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13
Q

Vit B12 def- essentials of dx

A
  • macrocytic anemia
  • megaloblastic blood smear (macro-ovalocytes and hypersegmented neutrophils)
  • low serum vit B12 level
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14
Q

Vit B12 def- sx

A
  • anemia
  • glossitis
  • peripheral n’s- paresthesias first, then balance or proprioception impairment
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15
Q

folic acid def- essentials of dx

A
  • macrocytic anemia
  • megaloblastic blood smear (macro-ovalocytes and hypersegmented neutrophils)
  • reduced folic acid levels in RBCs or serum
  • normal serum VitB12 level
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16
Q

lab features that suggest hemolysis

A
  • haptoglobin decreased (normally binds/clears hemoglobin)

- inc of indirect bilirubin

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17
Q

Paroxysmal Nocturnal Hemoglobinuria- essentials of dx

A
  • episodic hemoglobinuria
  • thrombosis is common
  • suspect in confusing cases of hemolytic anemia or pancytopenia
  • flow cytometry demonstrates deficiencies of CD55 and CD59
  • lysis of RBCs by complement
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18
Q

Paroxysmal Nocturnal Hemoglobinuria- sx, signs

A
  • reddish-brown urine

- prone to thrombosis- mesenteric, hepatic v’s, CNS veins, skin vessels

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19
Q

Paroxysmal Nocturnal Hemoglobinuria- lab

A
  • serum LD elevated
  • flow cytometry- CD55 and 59
  • FLAER assay- sensitive
20
Q

Paroxysmal Nocturnal Hemoglobinuria- tx

A
  • most pts have mild dz that doesnt require intervention
  • if severe- allogeneic HSC transplantation
  • Eculizumab (ab against complement protein C5)
21
Q

Glucose-6-P Dehydrogenase Deficiency- essentials of dx

A
  • X-linked recessive disorder (common in American black men)
  • episodic hemolysis in response to oxidant drugs or infection
  • Heinz bodies, bite cells and blister cells on PBS
  • reduced levels of G6PD b/w hemolytic episodes
22
Q

Glucose-6-P Dehydrogenase Deficiency- tx

A

-avoid oxidant drugs!!

23
Q

Sickle Cell Anemia- essentials of dx

A
  • recurrent pain episodes
  • FH and lifelong hx of hemolytic anemia
  • irreversibly sickled cells on PBS
  • Hemoglobin S is the major hemoglobin seen on electrophoresis
24
Q

Sickle Cell Anemia- signs, sx

A
  • chronic hemolytic anemia- jaundice, pigment gallstones, splenomegaly
  • vaso-occlusion crises- bones, chest
25
Q

Sickle Cell Anemia- lab dx

A

-hemoglobin electrophoresis- Hemoglobin S 85-98% of hemoglobin

26
Q

Sickle Cell Anemia- tx

A
  • hydroxyurea- reduces freq of pain crises
  • supportive care- mainstay!- folic acid supplementation
  • vaso-occlusive crises- exchange transfusions
27
Q

Autoimmune Hemolytic Anemia- essentials of dx

A
  • acquired hemolytic anemia caused by IgG autoab
  • spherocytes and reticulocytosis on PBS
  • positive antiglobulin (coombs) test
28
Q

Autoimmune Hemolytic Anemia- caused by?

A
  • idiopathic (50%)

- in assoc with SLE, CLL, or lymphoma

29
Q

Autoimmune Hemolytic Anemia- tx

A
  • prednisone
  • if ineffective- splenectomy
  • rituximab
30
Q

Cold Agglutinin Disease- essentials of dx

A
  • inc reticulocytes on PBS
  • antiglobulin (coombs) test positive only for complement
  • positive cold agglutinin titer
31
Q

Cold Agglutinin Disease- caused by?

A

-IgM autoab against an antigen on RBCs at lower temps- fixes complement- causes extravascular hemolysis

32
Q

Cold Agglutinin Disease- tx

A

-Rituximab

33
Q

Aplastic Anemia- essentials of dx

A
  • pancytopenia
  • no abnormal hematopoietic cells seen in blood or BM
  • hypocellular BM
34
Q

Aplastic Anemia- caused by?

A
  • autoimmune- idiopathic (most common), SLE
  • congenital- defects in telomere length maintenance
  • chemotherapy
  • toxins
  • drugs
  • pregnancy
  • PNH
35
Q

Aplastic Anemia- tx

A
  • mild- erythropoietic or myeloid GFs, RBC and plt transfusions
  • severe (neutrophil count <500, plts < 20,000, reticulocytes <1%, BM cellularity < 20%)- allogeneic BM transplantation (HLA-matched sibling)
  • adults > 40 without donor- immunosuppression with ATG (equine antithymocyte globulin) plus cyclosporine
36
Q

Acute intermittent porphyria- essentials of dx

A
  • unexplained abd crisis, generally in young women
  • acute peripheral or CNS dysfxn; recurrent psychiatric illnesses
  • hyponatremia
  • porphobilinogen in the urine during an attack
37
Q

Acute intermittent porphyria- types

A
  • presents in adulthood as AIP- inherited AD- mutation in HMBS
  • partial def of hydroxymethylbilane synthase activity- inc excretion of aminolevulinic acid and porphobilinogen in urine
  • characteristic abd pain- due to abnormalities in autonomic innervatoin in hte gut
38
Q

Acute intermittent porphyria- signs, sx

A
  • intermittent abd pain- neurologic!
  • peripheral neuropathy- can be mild or profound
  • CNS manifestations- seziures, altered consciousness, psychosi
39
Q

Acute intermittent porphyria- lab

A
  • hyponatremia
  • inc amt of porphobilinogen in urine during an acute attck
  • mutations in HMBS causing AIP- in 90% of pts
40
Q

Acute intermittent porphyria- prevention

A

Acute intermittent porphyria- prevention

41
Q

Acute intermittent porphyria- tx

A
  • high-carb diet

- acute attacks- tx with analgesics, IV glucose in saline and hematin

42
Q

Porphyria cutanea tarda- essentials of dx

A
  • noninfl blisters on sun-exposed sites, esp the dorsal surfaces of the hands
  • hypertrichosis, skin fragility
  • assoc liver dz
  • elevated urine porphyrins
43
Q

Porphyria cutanea tarda- what is it?

A
  • most common type of porphyria!

- assoc with ingestion of certain meds (estrogens) and liver dz from alcoholism, hemochromatosis or hep C

44
Q

Porphyria cutanea tarda- sx, signs

A
  • painless blistering and fragility of skin on hands

- facial hypertrichosis and hyperpigmentation is common

45
Q

Porphyria cutanea tarda- lab

A

-urinary uroporphyrins- elevated 2x-5x bove coproporphyrins

46
Q

Porphyria cutanea tarda- prevention

A

-barrier sun protection

47
Q

Porphyria cutanea tarda- tx

A
  • stop triggering medications
  • reduce alcohol consumption
  • most pts improve with treatment
  • phlebotomy without oral iron supplementation; can be with low-dose antimalarial medication

LHT2 (17 decks)

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