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HAEMATOLOGY/IMMUNOSUPPRESSION > PATHOLOGY > Flashcards

PATHOLOGY Flashcards

1
Q

Which immunoglobulin antibody is most associated w/ multiple myeloma?

A

IgG, which is produced by the high proliferation on monoclonal plasma cells underlying the neoplasia

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2
Q

What are the main functions on Von Willebrand factor?

A

Adhesion of platelets to collagen via glycoproteins
carrier molecule for factor VIII.

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3
Q

What is Fanconi anemia and what are its features?

A

an inherited cause of bone marrow failure

There is pancytopenia (decreased RBCs, WBCs, and platelets) along with abnormal skin (pigmented spots), bones (wide thumbs), and facial findings (small eyes),

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4
Q

What is paroxysmal noctural hematuria?

A

Paroxysmal nocturnal hematuria occurs due to abnormality in PIG-A gene, resulting in impaired synthesis in GPI anchors. This results in decreased extracellular complement inhibitors and leads to hemolysis.

presents with fatigue, abdominal pain, and dark-colored urine that classically resolves during the day

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5
Q

What are the features of hereditary spherocytosis?

A

In hereditary spherocytosis, the RBC membrane is not as flexible due to mutations in one of the genes that code for components of the RBC membrane/cytoskeleton, most commonly spectrin, ankyrin, band 3, and band 4.2.

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6
Q

What is the classic presentation of thrombotic thrombocytopenic purpura?

A

fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, and decreased renal function (elevated serum creatinine).

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7
Q

What are the features of disseminated intravascular coagulation?

A

activation of the coagulation cascade, which leads to deposition of microthrombi and consumption of platelets, fibrin, and coagulation factors, with subsequent bleeding

the deposition of fibrin in the microcirculation leads to a hemolytic anemia as RBCs are fragmented, which are schistocytes on the peripheral blood smear

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8
Q
  1. What features are associated w/ essential thrombocythemia?
  2. What laboratory values are consistent w/ this diagnosis?
A
  1. right-sided hemiparesis (suggesting a transient ischemic attack), bleeding from different parts of her body, burning and redness in her hands and feet, an enlarged spleen, and markedly increased platelets
  2. isolated elevation in platelet count along with splenomegaly and erythromelalgia, while other values are within normal ranges
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9
Q
  1. Which histopathologic finding is most diagnostic of primary myelofibrosis, and what process produces them?
  2. What is the pathophysiology of primary myelofibrosis?
  3. What are the clinical features of primary myelofibrosis?
A
  1. dacrocytes (teardrop-shaped RBCs) => result of physical damage sustained from the squeezing of RBCs out of increasingly fibrotic bone marrow.
  2. myeloproliferative disorder in which bone marrow is gradually replaced by collagen fibers.
  3. fatigue, anemia, splenomegaly, unsuccessful bone marrow retrieval
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10
Q
  1. What are the features of hereditary spherocytosis?
  2. How does hereditary spherocytosis affect hemoglobin?
A
  1. Hereditary spherocytosis is an autosomal-dominant anemia caused by defects in the RBC cytoskeleton or the proteins that attach it to the RBC membrane. Patients present with jaundice and splenomegaly.
  2. RBCs reveal an increased mean corpuscular hemoglobin concentration, as it becomes more prominent with the smaller size of the RBCs
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11
Q

What is the characteristic histologic feature for thalassemia?

A

target cells

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12
Q

Intravascular hemolytic anemias are characterized by

A

decreased serum haptoglobin
increased schistocytes on blood smear
anemia
elevated bilirubin

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13
Q

Extravascular hemolytic anemias are characterized by

A

spherocytes in peripheral blood smear
elevated bilirubin/bilirubin in urine

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14
Q

What are the extrinsic hemolytic anemias?

A

hereditary spherocytosis
paroxysmal nocturnal hemoglobinuria
G6PD deficiency
sickle cell anemia
pyruvate kinase deficiency

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15
Q

What are the features of hereditary spherocytosis?

A

autosomal dominant
defect in proteins interacting w/ RBC membrane skeleton (ankyrin, spectrin)
features include: splenomegaly, pigmented gallstones, aplastic crisis

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16
Q

What are the key histologic features of hereditary spherocytosis?

A

spherocytes

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17
Q

What are the features of paroxysmal nocturnal hemoglobinuria?

A

stem cell mutation –> increased intravascular hemolysis (especially at night)

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18
Q

What are the features of G6PD deficiency?

A

X linked recessive
G6PD defect leads to decreased NADPH (due to reduced oxidation), increased RBC susceptibility to oxidative stress = hemolysis
causes both intravascular + extravascular hemolysis

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19
Q

What are the key histologic findings in G6PD deficiency?

A

Heinz bodies + bite cells

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20
Q

What is the key extrinsic hemolytic anemia? and what are its features?

A

autoimmune hemolytic anemia
features include spherocytes + clumped RBCs

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21
Q

What is the key histologic finding of AML?

A

increased circulating myeloblasts
Auer rods in blasts

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22
Q

What is the key histologic finding of CLL?

A

crushed lymphocytes (smudge cells)

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23
Q

What is the key histologic finding of ALL?

A

increased lymphoBLASTS

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24
Q

What is this cell? What disease(s) is it present in?

A

target cells
Asplenia, liver disease, thalassemia

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25
Q

What is this cell? What disease(s) is it present in?

A

sickle cells
sickle cell anemia

26
Q

What is this cell? What disease(s) is it present in?

A

spherocytes
hereditary spherocytosis, autoimmune hemolytic anemia

27
Q

What is this cell? What disease(s) is it present in?

A

bite cells
G6PD deficiency

28
Q

What is this cell? What disease(s) is it present in?

A

schistocytes (helmet cells), which are fragmented RBCs
microangiopathic RBCs, hemolysis

29
Q

What is this cell? What disease(s) is it present in?

A

dacrocytes (teardrop cells)
myelofibrosis

30
Q

List the microcytic anemias and their broad categorizations

A

defective globin chains = thalassemia
defective heme synthesis = ACD, IDA, lead poisoning

31
Q

List the normocytic anemias and their broad categorizations

A
32
Q

What are the features of von Willebrand disease?

A

defect in intrinsic pathway due to decreased function of vWF
defect in platelet plug formation due to decreased platelet to vWF adhesion

33
Q

What does prothrombin time measure?

A

extrinsic + common pathway
1, 2, 5, 7, 10

34
Q

What does activated partial thromboplastic time measure?

A

intrinsic pathway + common pathway
factors I, II, V, VIII, X, XI, XII

35
Q

What is emergent treatment of vWD?

A

tranexamic acid
DDAVP (causes temporary surge of vWD)
Plasma derived clotting factor concentrate

36
Q

What are the features of hemophilias?

A

x linked recessive (males are affected), women are carriers

37
Q

What are the features of DIC?

A
38
Q
A
39
Q
A
40
Q

What test is used to detect antibodies bound to red blood cells in autoimmune hemolytic anemia?

A

direct coombs test

41
Q

What is the timeline of difference infections in bone marrow transplant infections?

A

0-30 days post transplant + preengraftment = immunosuppression + neutropenia + mucositis –> at risk of bacterial, systemic fungal
(candida, aspergillus) and viral (HSV) infections

30-100 days post engraftment = impaired cellular + humoral immunity, risk of graft vs host disease –> at risk of opportunistic bacterial infections + viral infections like CMV, EBV, hep B

1-2 years = impaired antibody mediated response = community respiratory viruses, encapsulated viruses, VZV

42
Q

What is the difference b/w multiple myeloma and MGUS?

A

MM = monoclonal plasma cells > 10%, predominantly IgG
MGUS = monoclonal plasma cells < 10%, any Ig

43
Q

An asymptomatic patient with a mild microcytic anemia and a normal RBC distribution width most likely has a form of ______ .

A

thalassemia

44
Q

___________ is immune-mediated destruction of platelets, commonly occurring in children weeks after an upper respiratory tract infection. It manifests as ecchymosis, petechiae, mucosal bleeding, and marked thrombocytopenia.

A

immune thrombocytic purpura

45
Q

What type of immunoglobulins are associated with cold autoimmune hemolytic anemias?

A

IgM antibodies, known as cold agglutinins, attach avidly to RBCs only in the colder locations of the body—such as the nose, lips, fingers, and toes. IgM antibodies cause agglutination of RBCs that leads to vasoocclusion and fingertip cyanosis.

46
Q

How does hydroxyurea function?

A

by inhibiting ribunucleotide reductase, which results in inhibiting of DNA synthesis and bone marrow suppression

47
Q

when is hydroxyurea used?

A

in proliferative diseases of the bone marrow

48
Q

Is electrophoresis alone is sufficient to detect monoclonal protein in the urine (or serum)?

A

No, Immunofixation is needed to determine whether a monoclonal light chain is present

49
Q

What are the criteria for multiple myeloma diagnosis

A

1.The presence of a serum or urine monoclonal protein
2. The presence of clonal plasma cells in the bone marrow or a plasmacytoma
3. The presence of end organ damage related to plasma cells including hyperCalcemia,
Renal dysfunction, Anaemia and/or lytic Bone lesions = CRAB

50
Q

What are the criteria for monoclonal gammopathy diagnosis

A
  1. The serum monoclonal protein is < 3 g/dL
  2. Bone marrow plasma cells are < 10%
  3. No end organ damage is present (no CRAB)
51
Q

What is the key feature of acute promyelocytic leukemia?

A

APML is associated with a t(15;17) translocation, causing failure of immature myeloid precursors (myeloblasts) to differentiate into mature granulocytes

52
Q

What is the function of Bcr-2?

A

Bcl-2, blocks apoptosis and promoting growth of the malignant cells

53
Q

What malignancy is associated with Bcr-2

A

B cell lymphoma
follicular lymphoma

54
Q

What is the role of transferrin?

A

Transferrin is a protein that binds iron in the blood and transports it to various tissues, including the bone marrow, where it is used for red blood cell production.

55
Q

What does high transferrin levels indicate?

A

Indicates increased transferrin levels and suggests that the body is iron-deficient.

56
Q

What is aplastic anemia?

A

failure/destruction of stem cells in bone marrow leading to reduced reticulocyte count and increased EPO (due to decreased erythropoiesis)

57
Q

What are the laboratory values associated with aplastic anemia?

A

pancytopenia
normal cell morphology –> hypocellular bone marrow w/ fatty infiltration

58
Q

Invasive aspergillosis is treated with ___

A

voriconazole

59
Q

Invasive candidiasis is treated with __

A

amphotericin B or echinocandins

60
Q

What are the four F’s of HIV infection?

A

flu like
feeling fine
falling count
final crisis

61
Q

What protozoal infections are most common in the immunocompromised?

A

toxoplasma
pneumocystic jirovecii

62
Q

What fungal infections are most common in the immunocompromised?

A

candida
aspergillus
cryptococcus

HAEMATOLOGY/IMMUNOSUPPRESSION (3 decks)

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