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STEP 1 STUFF > Pathoma Whit Blood Cell Disorders > Flashcards

Pathoma Whit Blood Cell Disorders Flashcards

1
Q

Hematopoeitic Stem Cell

A

CD 34

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2
Q

GMCSF/GCSF

A

can be used to treat neutropenia, can boost granulocyte production.

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3
Q

Causes of neutropenia

A

Drug toxicity - chemptherapy

Sepsis - neutrophils have moved in to the tissue, low circulating amount in the plasma

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4
Q

Lymphopenia

A

DiGeorge syndrome - athymic
High Cortisol state - Induces apoptosis of the lymphocytes
Autoimmune destruction - i.e. secondary to SLE
Whole body radiation - lymphocyte is the most sensitive cell in the human body to radiation.

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5
Q

Neutrophilic leukocytosis

A

bacterial infection
- will have left shift, immature precursors released in to the blood. These cells are characterized by decreased Fc receptors. thus they don’t function as well as mature cells. CD16 is the marker for Fc receptor.

tissue necrosis

high cortisol state - from demargination due to cortisol disrupting adhesion.

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6
Q

Monocytosis

A

seen in chronic inflammatory states and malignancy

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7
Q

Eosinophilia

A

seen in allergic reactions, parasitic infections.

and a subset of patients with Hodgkins lymphoma. Due to increased IL-5 production.

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8
Q

Basophilia

A

Classically associated with CML

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9
Q

Lymphocytosis

A

CD 8+ T-cells to attack the viral infection.

Remember the association with bordetella pertusis. This bacteria produces a lymphocytosis promoting factor. It prevents the lymphocytes from leaving the blood to enter the lymph node leading to lymphocytosis on labs.

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10
Q

Infectious mononucleosis LAD - which are of the lymph node is enlarged?

A

Paracortex

Cortex is the home of the bcells, in IM the response is CD8 T-cell

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11
Q

Why is there splenomegaly in Infectious Mononucleosis

A

Like lymph node, expansion of T-cell area of white pulp, the periarterial lymphatic sheath.

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12
Q

Monospot Test

A
  • Detects IgM heterophile antibodies - affinity to bind RBCs of anther animal (i.e. sheep or horse).
  • Positive 1 week after infection
  • WILL BE NEGATIVE when the disease is due to CMV.

Monospot is a screening test.
Definitive diagnosis is antibody to EBV viral capsid antigen.

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13
Q

Infectious Mono Complications

A
  • splenomegaly
  • rash with penicillin
  • virus can be dormant in BCell, disease can recur. and there is a risk of developing lymphoma in the future. Especially in patients with immunodeficiency.
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14
Q

Leukemia definition

A
  • a proliferation of blasts. Greater than 20 percent blasts in the bone marrow.
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15
Q

Presentation

A

Loss of other cells normally produced by bone marrow due to “crowding out by blasts”. I.e. anemia, thrombocytopenia, neutropenia.
- typically an acute presentation.

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16
Q

Blasts appearance on smear

A
  • Large compare to RBC

- Look for nucleolus (punched out area)

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17
Q

Key marker for lymphoblast?

A

TdT in the nucleus. A DNA polymerase present only in lymphoblasts (not present in mature lymphocytes and not present in myeloblasts)

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18
Q

Key marker for myeloblast?

A

myeloperoxidase

- can crystalize into auer rod and this can be appreciated under the microscope.

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19
Q

ALL

A

commonly arises in children

associated with down syndrome, but usually after the age of 5.

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20
Q

B-ALL vs T-ALL

Surface markers

A

distinguish via surface markers. BOTH will be TdT positive.
B-ALL: CD 10, CD19, CD20
T-ALL: CD2 up to CD8. They DO NOT experss CD 10.

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21
Q

B-ALL treatment

A

has excellent response to chemo. remember to include prophylaxis to the scrotum and CSF.

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22
Q

Cytogenic abnormalities in B-ALL

A

t(12,21) - most common in children. good prognosis.
T(9,22) - poor prognosis. usually seen in adults. REMEMBER this the characteristic finding of CML, but can present in this subset of adults with B-ALL.

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23
Q

T-ALL presentation

A

Thymic mass
Teenager
- We usually call this acute lymphoblastic lymphoma because it forms a mass instead of the cells being in the blood as with B-ALL.

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24
Q

AML

A
  • older adults
  • look for MPO, stain or auer rods
  • look for cytogenic abnormality. If there is not one present then characterized by differentation lineage.
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25
Q

Acute Promyelocytic Leukemia

A

t(15,17)

  • this results in disruption the reitnoic acid receptor halting cell growth, so blasts proliferate.
  • Treatment is ATRA, all trans retinoic acid. This will cause the cells to mature to neutrophils thus alleviating the blast burden.
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26
Q

Acute Monocytic Leukemia

A
  • Proliferation of monoblasts

- Characteristically infiltrate the gums

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27
Q

Acute megkaryoblastic leukemia

A
  • Lack MPO

- associated with down syndrome before the age of 5.

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28
Q

Patients with down syndrome and associated leukemia risk:

A

acute myelblastic leukemia before age 5

acute lymphocytic leukemia after the age of 5

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29
Q

Myelodysplastic syndrome

A

Bone marrow biopsy would show increased blasts but less than 20 percent. The cells are not properly formed. They do not get out in to the blood. Patients present ith cytopenia.

NOTE - may progress to acute leukemia

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30
Q

Chronic Leukemia

A

Proliferation of MATURE lymphocytes.
Insidious in onset, present with high WBC and are foten asymptomatic.
Classically seen in older adults.

31
Q

CLL - markers

A

neoplastic proliferation of naive B-Cells

- coexperssion CD5 and CD20

32
Q

CLL blood smear

A

Increased # of lymphocytes and smudge cell.

33
Q

Complications of CLL

A
  • hypogammaglobulinemia
  • autimmune hemolytic anemia
  • trasnformation to diffuse large B-Cell lymphoma
34
Q

Hairy Cell Leukemia

A

Proliferation of mature B-Cells.

+ for tartrate resistant acid phosphotase

35
Q

Clinical features of hairy cell leukemia

A
  • dry tap, bone marrow fibrosed
  • enlarged spleen due to expansion of RED PULP.
  • NO lymphadenopathy
36
Q

Treatment for hairy cell leukemia

A

2-CDA inhibits adenosine deaminase leading to death of the neoplastic B-Cells.

37
Q

Adult T-Cell Leukemia/Lymphoma (ATLL)

A
  • associated with HTLV-1 virus.
  • common in Japan and the caribbean
  • Proliferation of mature CD4+ T-cells
38
Q

Adult T-Cell Leukemia/Lymphoma (ATLL) Clinical Presentation

A

RASH, generalized LAD w/ HSM, lytic punched out bone lesions with hypercalcemia.

39
Q

Mycosis fungoides

A

Proliferation of mature CD4+ T-cells in the skin. T-cells in epidermis on biopsy –> called Pautrier microabscess.

40
Q

Sezary syndrome

A
  • when mycosis fungoides spreads to the blood.

CHARACTERISTIC cerebriform nuclei.

41
Q

Myeloproliferative Disorders

A

Neoplastic over production of mature myeloid lineage. Note that ALL MYELOID LINES ARE INCREASED, but the disorder is named for the predominant cell being over produced.

  • High white blood cell count with hypercellular marrow.
42
Q

Common complication of myeloproliferative disorders

A
  • increased risk of gout and hyperurecemia due to increased cell turnover.
  • marrow fibrosis
  • can transform to acute leukemia
43
Q

Chronic Myeloid Leukemia

A
  • Increase in granulocytes. Basophils are characteristically increased.
  • t(9,22) leading to BCR-ABL fusion with increased tyrosine kinase activity.
44
Q

CML treatment

A

Imatinib - blocks tyrosine kinase activity

45
Q

CML transformation to acute leukemia

A

AML or ALL

46
Q

CML vs Leukomoid Reaction

A
  • CML granulocytes are LAP (leukocyte alkaline phosphatase) negative
  • CML is associated with increased basophils
  • CML granulocytes exhibit t(9,22)
47
Q

Polycythemia Vera

A

Driven by JAK-2 kinase mutation

48
Q

Polycythemia Vera Clinical Signs and Symptoms

A

Mostly due to hyperviscosity of the blood

  • blurry vision and headache
  • thrombosis (especially budd-chiari syndrome)
  • flushed face
  • itching after bathing due to histamine release from increased mast cells.
49
Q

Polycythemia Vera Treatment

A
  • phlebotomy

- 2nd line: hydroxyurea

50
Q

Distinguishing PV from reactive polycythemia

A

In lung disease - EPO would be high, SaO2 would be low.
In RCC producing EPO, EPO would be high and SaO2 would be normal

In PV - EPO would be decreased due to negative feedback. SaO2 normal.

51
Q

Essential Thrombocythemia

A

Also associated with JAK2 kinase mutation.

52
Q

Lymphadenopathy

A

painful - acute infection, the lymph node draining that region of infection.

painless - differential includes chronic inflammation (autoimmune), metastatic carcinoma (i.e breast cancer to axillary lymphnodes), lymphoma.

53
Q

Lymph node hyperplasia by region of the lymph node
Follicle
Paracortex
Sinus Histiocytes

A

Follicle - RA, early HIV.

Paracortex - viral infection

Sinus Histiocytes - lymph node draining tissue with cancer

54
Q

Lymphoma definition

A
  • neoplastic proliferation of lymphoid cells that forms a mass.

Divided into non-hodgkins lymphoma (60%) and hodgkins

55
Q

Follicular lymphoma

A

t(14,18) CD 20
- translocation puts BCL2 from chromosome 18 into the position of IgG on chromosome 14, highly active area in B-Cells. BCL2 suppresses apoptosis leading to overproduction of B-Cells.
make follicle like nodules
presents in late adult hood with enlargement of the lymph nodes.

56
Q

Follicular lymphoma treatment

A

rituximab (anti CD20 antibody) or low dose chemo

most patients are asymptomatic, no treatment necessary.

57
Q

Follicular lymphoma complications

A

Enlarging lymph nodes - concern for progression to diffuse large B-Cell lymphoma.

58
Q

Follicular Lymphoma VS Follicular hyperplasia in response

A

Lymphoma:

  • loss of normal follicular architecture
  • lack of tingible macrophages in the germinal center
  • monclonality
  • Expression of Bcl 2
59
Q

Mantle Cell Lymphoma

A

T(11,14) translocation. Cyclin D from 11 moved to 14 spot of IgG heavy chain.
Neoplastic B cells CD 20 positive, small cell.
- cyclin allows the cell to go to from G1 - S, so this translocation leads to aberrant growth.
Proliferation of small B-cells expanding the mantle zone (expansion of region immediately adjacent to the follicle).

60
Q

Marginal Zone Lymphoma

A

expansion of the marginal zone. neoplastic B-cells CD 20 positive. small cell.

61
Q

Disorders associated with Marginal Zone Lymphoma

A
  • Sjogrens, hashimotos thyroiditis, H Pylori gastritis

–> H Pylori gives gastric MALToma.

62
Q

Burkitt Lymphoma

A

t(8,14) translocation results in trasnlocation of c-myc on 8 to heavy chain locus on 14. C-myc is an oncogene that promotes growth.

Classic presentation: extranodal mass in a child or young adult. the african form produces a jaw mass. the sporadic form produces a mass in the abdomen.

INTERMEDIATE sized cell.

63
Q

Burkitt Lymphoma on histology

A

Starry sky appearance due to high mitotic activity.

64
Q

Diffuse Large B-Cell

A
  • most common non-hodgkins lymphoma. poorly differentiated. presents in late adult hood usually as enlarging lymph node, but can be extranodal mass.
65
Q

Hodgkins Lymphoma

A

Reed Sternberg Cells - secrete cytokines drawing in inflammatory cells that result in the production of a mass.

These cells are usually CD 15 and CD 30 positive.

Notice that they are not CD 20 positive, but are B-cells.

66
Q

Nodular sclerosis subtype of Hodgkins

A
  • will see fibrosis on histology. It is the most common type of Hodgkins lymphoma.
67
Q

Multiple Myeloma

A

Most common primary malignancy of bone (but less commonly seen in the bone than mets).
A malignant proliferation of plasma cells in the bone marrow.
IL-6 is often elevated in these patients (it is an important growth factor for plasma cells).

68
Q

Signs and symptoms of Multiple Myeloma

A
  • Osteoclast activating factor is produced. activates RANK receptor on osteoclasts leading to bone degradation.
  • Punched out lesions seen on X-ray (vertebrae and skull specifically)
  • Also will hypercalcemia

-Elevated serum protein. M-spike on Serum Protein Electrophoresis (SPEP). M-spike indicates monoclonal immunoglobulin. (it is most commonly IgG or IgA).

69
Q

Multiple Myeloma complications

A
  • Monoclonal immunoglobulin leads to loss of antigenic diversity. Increase risk for infection. Infection being the most common cause of death in multiple myeloma.
  • Primary AL amyloidosis from high circulating levels of light chains.
  • When light chain is secreted in the urine it is secreted as bence jones protein.
  • deposition of circulating light chain proteins in the kidney itself results in renal failure.
70
Q

Multiple Myeloma finding on blood smear

A

Rouleaux formation

71
Q

MGUS

A

M-Spike without the other findings of multiple myeloma. Commonly in the elderly –> 5 percent over 70 have MGUS.

There is a risk of going on to developing multiple myeloma. about 1 percent each year.

72
Q

Waldenstrom Macroglobulinemia

A

increased serum IgM. This large Ig increases viscosity of the blood.

73
Q

Langerhans Cell Histiocytosis on histology

A

tennis racket granule (Birbeck granule)

STEP 1 STUFF (15 decks)

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