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Flashcards in Anemias/Leukemias Deck (79)
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31

Compare thalassemia minor and thalassemia major.

Thalassemia minor = 1 deletion, mild anemia (trait)
microcytic, hypochromic
*note: looks like iron deficiency!!

Thalassemia major = hemolysis accompanied by splenomegaly, hemosiderosis, hepatomegaly, BM compensatory hyperplasia

32

Why are RBCs round in hereditary spherocytosis?

There are mutations in the genes encoding that proteins that make up the membrane skeleton. 

33

What is immune hemolytic anemia?

Recall: four blood groups = A, B, AB, O

 

Autoimmune hemolytic anemias develop as a consequence of an immune reaction to RBC autoantigens or neoantigens formed between the body's own proteins and hapten

 

34

Explain the pathogenesis of immune hemolytic anemia.

Hemolytic anemias:

 

1. Autoantigens present on RBCs are normally not recognized as foreign by the body. In some persons the body produces antibodies to its own antigens on RBCs. This occurs for no obvious reasons.

2. Alloantigens are foreign antigens. For example, blood group B RBCs are recognized as foreign by group A persons. 

3. Neoantigens are from from the body's own protein's linked to a non-immunologic hapten. IgG, immunoglobulin G. 

35

Compare primary and secondary polycythemia.

Primary = clonal proliferation of hematopoietic stem cells and increased total RBC mass (neoplastic)

 

Secondary = increased RBC volume as a result of erythroid bone marrow hyperplasia caused by erythropoietin

36

What is leukopenia, and what are its causes?

leukopenia = reduction in the WBC count

 

Can be caused by:

drugs

chemicals

radiation

aplastic anemia

37

What is leukocytosis, and what are its causes?

leukocytosis = increased WBCs (>10,000)

 

Causes:

reactive luekocytosis (splenomegaly in response to bacterial infection)

lymphoma

leukemia

38

What are the possible causes of lymph node enlargement?

  • common in children with URI
  • with EBV
  • early stages of AIDs
  • most common presenting symptom of lymphoma

39

What is the difference between lymphoma and leukemia?

Lymphoma = lymphoid cell malignant disease prodominantly involving the lymph nodes

 

Leukemia = increased number of malignant WBC in the peripheral blood due to WBC precursors in the BM

40

What causes lymphomas and leukemias?

Most causes are unknown. 

viruses = HTLV-1, EBV

oncogenes = translocation (Burkitts), philadelphia chromosome (CML), hybrid BCR, ABL

41

What are the common features of all leukemias?

  1. BM is infiltrated with malignant cells
  2. Peripheral blood contains increased immature cells
  3. Chromosomal and genetic changes specific for each disease
  4. Complications: anemia, recurrent infections, uncontrollable bleeding

**INFECTION = most common cause of death in all forms of leukemia

42

What distinguishes acute from chronic leukemia and lymphocytic from myelogenous leukemia?

43

List the most important features of acute lymphoblastic leukemia and correlate the pathologic findings with the clinical features of this disease.

  • massive BM infiltration with immature lymphoid cells (blasts = T and B precursors)
  • most common form of leukemia in children, and most common malignant disease in children <5yrs
  • rapid course, marked recurrent infections, generalized weakness, bleeding into the skin and internal organs
  • remission can be induced, 2/3 are CURED

44

List the most important features of acute myelogenous leukemia.

Clonal proliferation of myeloblasts in the bone marrow.  Must find 20% in the BM biopsy! 

  • Most common form of acute leukemia in adults.
  • Treatment: high dose radiation and chemo. 

45

List the most important features of chronic myelogenous leukemia.

Malignant disease of pluripotent stem cells capable of differentiating into neutrophilic leukocytes. 

  • Mostly affects ADULTS
  • SLOW onset; anemia, SM, thrombosis
  • Tx: radiation + chemo = 70% 3 yr survival

46

3 phases of CML

  1. chronic phase: marked leukocytosis, increased eosinophils and basophils. BM <10 % blasts
  2. accelerated phase: BM >10% blasts, >20% basophils in peripheral blood.
  3. blast crisis: BM >20% blasts

47

90% of patients have the Philadelphia chromosome with BCR-ABL gene arrangement . . . for which leukemia???

CML

48

Do you have worse or better prognosis when you have the Philadelphia chromosome?

BETTER!!!

49

List the most important features of chronic lymphocytic leukemia.

Malignant disease of lymphoid cells.

  • Disease of OLDER age (>50)
  • SLOW course
  • Sx = lymphadenopathy and peripheral leukocytosis
  • Tx: cells do not grow rapidly, therefore NO CHEMO
  • can TRANSFORM into more aggressive form

50

How do you diagnose CLL?

CLL cells look NORMAL on a smear!!  Suspect if lymphocytes >5000 (Nl = <4000). BM Biopsy confirms diagnosis. 

51

How are non-Hodgkin’s lymphomas classified?

Two categories:

  • B-cell
  • T-cell and NK cell 

52

True or False:

All lymphomas are malignant

True

53

True or False:

Most lymphomas have a B-cell phenotype

TRUE

54

Extranodal sites for lymphomas are . . .

can be outside of the lymph nodes, like eyes, brain, skin. 

Most common is the GI system!

55

Immunohistochemistry

Uses antibodies to analyze tissues for tumor cells. 

Can help determine B and T cell and zones of the lymph node (cortical, mantle, germinal center)

56

What method is used to determine maturation and phenotype of lymphocytes?

flow cytometry 

57

List the most common symptoms and clinical findings of non-Hodgkin’s lymphomas.

  • painless lymphadenopathy
  • systemic constitutional symptoms
  • extranodal spread

58

What are systemic constitutional symptoms?

  • fatigue
  • malaise
  • fever
  • weight loss
  • pruritis
  • sweating

59

What is the most common form of lymphoma in the UNited states?

Follicular lymphoma

60

Follicular Lymphoma

The follicular structure of lymph nodes is preserved. 

  • SLOW growing
  • Sx: long term lymphadenopathy, mild constitutional sx
  • Tx: does not response well to chemo