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Step 1 > Heme > Flashcards

Heme Flashcards

1
Q

Hemolytic Uremic Syndrome

A

-caused by shiga toxin producing bacteria (EHEC, shigella)
-features: prior bloody diarrheal illness, hemolytic anemia with schistocytes, thrombocytopenia (low platelets), acute kidney injury
-normal coagulation studies
Toxin damages capillaries, leading to platelet activation and formation of microemboli, that shear RBCs and cause schistocytes.
Causes low haptoglobin.
Dec. hemoglobin and platelet count.
Inc. lactate dehydrogenase, unconjugated bili, BUN, and creatinine.

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2
Q

Schistocytes

A
  • fragmented RBCs
  • diagnostic of a traumatic mechanism and indicate either microangiopathic hemolytic anemia (ex. HUS, TTP, DIC) or mechanical damage
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3
Q

Microangiopathic Hemolytic Anemia

A

-mechanism: formation of fibrin (protein) mesh in vessels that physically cuts the RBCs
-causes RBC schistocytes
-ex. HUS, DIC, TTP
May have dec. platelets due to micro vascular thrombi formation

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4
Q

Pernicious Anemia

A
  • often due to B12 deficiency/lack of intrinsic factor
  • fewer but larger RBCs
  • antibodies to intrinsic factor
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5
Q

Effects of CO Poisoning

A
  • inc. carboxyhemoglobin (HbCO)
  • no change in paO2 (amount of dissolved oxygen in plasma)
  • dec. O2 content and O2 carrying capacity
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6
Q

Von Willebrand Disease

A
  • most common inherited bleeding disorder
  • autosomal dominant
  • variable penetrance
  • absence of von willebrand factor causes impaired platelet function and coagulation issues due to dec. factor VIII activity
  • inc. PTT (may be unaffected) and inc. bleeding time
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7
Q

Hemophilia A

A
  • x linked
  • deficiency of factor 8
  • prolonged PTT
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8
Q

Hemophilia B

A
  • x linked
  • deficiency of factor IX
  • prolonged PTT
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9
Q

Vitamin K

A
  • required for factors II, VII, IX, and X

- prolonged PT and PTT

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10
Q

T-ALL

A
  • most often in teenagers
  • immature T cells migrate to thymus and cause mass
  • acute
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11
Q

Chronic Lymphoid Leukemia

A
  • chronic
  • lymph cells- B cells
  • smudge cell (ruptured fragile B cells on microscopy)
  • generalized lymph adenopathy (small lymphocytic lymphoma)
  • can lead to diffuse B cell lymphoma (actual mass of B cells in lymph node)
  • hypogammaglobulinemia (too few antibodies)
  • dysfunctional antibodies cause autoimmune hemolytic anemia (they attack RBCs)
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12
Q

Granulocytes

A
  • eosinophil
  • neutrophil
  • basophil
  • mast cell
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13
Q

Polycythemia Vera

A
  • Myeloproliferative disorder with uncontrolled erythrocyte production (RBC cancer)
  • inc. in all cell lines but esp. erythrocytes (high hematocrit, low EPO)
  • JAK2 mutation (non receptor tyrosine kinase associated with the erythropoietin receptor)
  • pruritis when hot
  • inc. bone marrow sensitivity to growth factors
  • tx: blood letting
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14
Q

Methemoglobinemia

A
  • dusky discoloration of skin
  • unable to carry oxygen
  • partial pressure of O2 (P02) will remain unchanged (measure of dissoved O2 and unrelated to hemoglobin)
  • can be caused by poisoning with nitrites, drugs
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15
Q

Carbon monoxide affects hemoglobin by:

A

Competitively binding to heme.

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16
Q

Von Willebrand Disease

A

Autosomal dominant. Causes impaired platelet adhesion with exposed dcollagen. VW also acts as a carrier for factor VIII.
Prolonged PTT and bleeding time.
Sx: heavy periods, and nose bleeds.

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17
Q

Haptoglobin

A

Binds free hemoglobin and thus haptoglobin is decreased when there is RBC damage.

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18
Q

Rouleaux Cells

A
  • stacked RBCs

- think multiple myeloma

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19
Q

Basophilic Stippling

A
  • reticulocytosis or lead poisoning

- cytoplasmic remnants of RNA

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20
Q

Howell Jolly Bodies

A
  • remnants of nuclear chromatin

- severe anemia or pts without spleen

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21
Q

Ring Sideroblast

A
  • iron trapped in mitochondria
  • form ring around nucleus
  • seen in sideroblastic anemia
  • seen in bone marrow not peripheral blood smear
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22
Q

Heinz Bodies

A
  • denatured hemoglobin (unstable heme like in H disease thalassemia)
  • can be seen in G-6-PD deficiency (African and Mediterranean type)
  • visualized with supravital stains (methylene blue, crystal violet)
  • eaten by macrophages to form bite cells
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23
Q

Causes of Microcytic Anemia (

A
  • iron deficiency
  • thalassemia
  • anemia of chronic dz
  • siderblastic anemia
  • lead poisoning
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24
Q

Causes of Macrocytic Anemia (>100 fL/cell)

A
  • megaloblastic anemia (B12 def, folate def)

- alcoholic liver dz (neutrophils 3-5 on peripheral blood smear)

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25
Q

Causes of Normocytic Anemia with low Reticulocyte Count

A
  • marrow failure
  • aplastic anemia
  • myelofibrosis
  • leukemia/metastasis
  • renal failure
  • anemia of chronic disease
  • should be inc. erythrocytes in anemia (check bone marrow)
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26
Q

Causes of Normocytic Anemia with High Reticulocyte Count

A
  • sickle cell
  • G6PD deficiency
  • hereditary spherocytosis
  • autoimmune hemolytic anemia
  • paroxysmal nocturnal hemoglobinuria
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27
Q

Fe Deficiency Anemia

A
  • dec MCV
  • dec MCHC
  • dec. RDW
  • inc. free erythrocyte protoporphyrin (FEP)
  • oral apithelial atrophy if Plummer Vinson syndrome present
  • koilonychia (spoon nails)
  • pica (eating stuff)
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28
Q

Sideroblastic Anemia

A
  • lead poisoning
  • B6 deficiency (drug like isoniazid)
  • alcoholism
  • myelodisplastic syndrome
  • ringed sideroblasts present in bone marrow
  • body has adequate iron but unable to incorporate it
29
Q

HbF

A
  • fetal hemoglobin contains y globulin (gamma)
  • composed of 2a and 2y (gamma) chains
  • babies with B thalassemia do not present at birth due to y globulin
  • made in fetus not embryo
30
Q

Which cells are tdt +?

A

-immature lymphcytes

31
Q

CD5 + CD 19 or 20?

A

-B chronic lymphocytic leukemia cells

32
Q

CD11c or CD 25 or CD 103 with CD19/20?

A
  • hairy cell leukemia

- dry tap with big spleen

33
Q

Burkitts

A
  • starry sky (macrophages eating tumor)
  • African kid with jaw mass or AIDS pts
  • t(8;14) cMYC to immunoglobulin heavy chain juxtaposition
34
Q

Mantel Cell

A
  • B cell lymphoma
  • mantel zone B lymphocytes (CD 5, 19, 20 +, CD23-)
  • t(11;14) CCD1 (cyclin D 1 protein)
35
Q

MALToma

A
  • chronic inflammation (H. pylori, Hashimoto, sjogren) to MALToma
  • lymphoma
36
Q

Multiple Myeloma

A
  • bone marrow tumor
  • inc. serum protein with normal albumin
  • M spike on serum electophoresis (most common is IgG or IgA)
  • rouleax cells
  • lytic bone lesions = bone pain, hypercalcemia, bone fracture
  • Bence Jones proteins are light chains filtered into urine
37
Q

Monoclonal Gammopathy of Undetermined Significance

A

-M spike with no bone lytic lesions or pain (as seen in multiple myeloma)
-may progress to multiple myeloma
-

38
Q

Adult T Cell Lymphoma

A
  • see in Japan and Caribbean
  • T cell tumor
  • skin lesions
  • lymph involvement
  • hypercalcemia
  • hyperlobated 4 leaf clover appearance
39
Q

Reed Sternberg Markers

A

-CD15+, CD30+, CD45-

40
Q

t(12;21)

A
  • ALL

- good prognosis with this translocation

41
Q

Auer Rods

A

-characteristic of Acute Promyelogenous Leukemia
Stain positive for myeloperoxidase.
Indicate myeloid lineage.

42
Q

What cancer can be treated with vitamin A?

A
  • PML
  • t(15;17)
  • auer rods
  • fusion of PML/RARA
  • RARA is vit A receptor and high vit A helps mature the cancer cells
43
Q

Essential Thrombocythemia

A
  • inc in all cell lines but esp. thrombocythemia

- JAK2 mutation

44
Q

Myelofibrosis

A
  • marrow fibrosis secondary to factors released from megakaryocytes
  • splenomegly
  • dry tap
  • biopsy: myelofibrosis and myosis
  • tear drop RBCs
  • JAK2 mutation
  • distinguish from hair cell leukemia and aplastic anemia
45
Q

Transferrin is (blank) in states of low iron.

A

Increased.

46
Q

Labs for Vitamin B12 Deficiency

A

High homocysteine and high methylmalonic

47
Q

Labs expected for folate deficiency.

A

Just inc. homocysteine, and normal methylmalonic acid.

48
Q

Uremic Platelet Dysfunction

A
  • inc. bleeding time with normal coagulation studies and normal platelet count
  • due to significant renal dysfunction due to accumulation of uremic toxins in circulation that impair platelet aggregation and adhesion
  • improved with dialysis
49
Q

Hereditary Spherocytosis

A
  • red cell cytoskeleton abnormalities
  • likely defect in spectrin or ankyrin
  • inc. osmotic fragility on acidified glycerol lysis testing confirms diagnosis
  • sx: hemolytic anemia, jaundice, splenomegaly
  • complications: pigmented gallstones and aplastic crises from parvovirus B19
50
Q

Henoch Schonlein Purpura

A
  • systemic hypersensitivity dz that produces leukoclastic angiitis in small vessels of the dermis and GI tract
  • purpuric rash, abdominal pain, and polyarthralgia
  • may cause acute glomerulonephritis
  • boys aged 3-11
  • associated with IgA and C3 deposition
51
Q

Paroxysmal Nocturnal Hemoglobinuria

A

-hemolytic anemia, hemoglobinuria which may be nocturnal
-thrombosis
Hypercoagulability
-pancytopenia
-complement mediated cell lysis
-chronic lysis may lead to hemosiderosis of kidney (iron deposition)
-mutated PIGA gene that helps attach many cell surface proteins (CD55, CD59) that inactivate complement

52
Q

Erythrocytosis

A
  • inc. in erythrocyte production

- this can be due to a left shift in the O2 dissociation curve, leading to hypoxia in the kidney, and inc. EPO synthesis

53
Q

Glanzmann Thrombasthenia

A

-deficient GP2b3a

54
Q

Acanthocytes

A

Irregular projections on RBCs, typical in abetalipoproteinemia

55
Q

Bite Cells

A

When spleen removes Heinz bodies. Due to glucose six phosphate dehydrogenase causing oxidant induced damage.

56
Q

Tear Drop Cells

A

Due to myelofibrosis. When marrow is fibrotic, cells must squeeze through.

57
Q

Hematocrit

A

-proportion of total blood volume that is composed of RBCs

58
Q

Thrombotic Thrombocytopenic Purpura

A
  • dec. ADAMT13 leads to large vWF multimers
  • pentad of fever, neurologic sx, kidney disease, microangiopathic hemolytic anemia, and thrombocytopenia, although not all pts have neuro sx or fever
  • is a primary thrombotic microangiopathy
  • thombi in arteriolar microvasculature, causing dec. platelets and schistocytes
  • tx: plasma exchange therarpy
59
Q

How to Identify Reticulocytes

A
  • immature RBC
  • larger and bluer than mature RBC
  • lacks nucleus
  • retains ribosomal RNA
  • appears blue on Wright-Giemsa Stain
60
Q

Pure Red Cell Aplasia

A
  • rare form of marrow failure
  • severe hypoplasia of marrow erythroid elements with normal granulopoiesis and thrombopoiesis
  • ass. with thymoma, lymphocytic leukemias, and parvovirus B19
61
Q

Immune Thrombocytopenic Purpura

A
  • immune mediated consumption of platelets
  • low platelet count and petechiae
  • inc. platelet cell production in bone marrow (not production issue)
62
Q

Sickle Cell Mutation

A
  • single nucleotide mutation on the B-globin gene
  • glutamic acid to valine
  • in low O2 concentrations, causes polymerization of HbS itself
63
Q

B12 Deficiency Signs

A
  • elevated MMA and homocysteine are characteristic
  • megaloblastic anemia
  • pernicious anemia (previous autoimmune disease likely)
64
Q

Factor V Leyden Deficiency

A
  • most common cause of hereditary hypercoagulability
  • factor V that is resistant to degradation by activated protein C
  • leads to inc. clotting
65
Q

Antithrombin Deficiency

A
  • no direct effect on PT, PTT, or thrombin time
  • diminishes inc. in PTT following heparin administration
  • can also be acquired in nephrotic syndrome if lost in urine
66
Q

Protein C or S Deficiency

A
  • dec. ability to inactivate factors Va and and VIIIa
  • inc. risk of thrombotic skin necrosis w/ hemorrhage following admin of warfarin
  • C Cancels Coagulation
  • protein C and S prevent coagulation
67
Q

Hemoglobin A1 vs A2

A
  • hemoglobin A= 2 alpha 2 beta

- hemoglobin A2= 2 alpha and 2 delta chains

68
Q

Glucose 6 Phosphate Dehydrogenase Deficiency

A
  • X linked recessive
  • more common in people of Mediterranean or African origin
  • may protect against malaria
  • rate limiting enzyme in production of NADPH
  • may be exacerbated by drugs, food (fava beans), or illness
69
Q

Prussian Blue Stain

A
  • identifies hemosiderin (iron overload)
  • hemosiderin is typically seen in kupffer cells in liver
  • hemosiderin laden macrophages = siderophages

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