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PATHOMA > 06-White blood cell disorders > Flashcards

06-White blood cell disorders Flashcards

1
Q

when does neutropenia occur?

A

1-in drug toxicity

2-in infection

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2
Q

what are causes of lymphopenia

A

immunodeficiency
high cortisol level
whole body radiation

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3
Q

which cells are most sensitive to radiation

A

lymphocytes

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4
Q

What is neutrophilic leukocytosis?

A

It is an increase in WBC count.

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5
Q

What causes neutrophilic leukocytosis?

A

Bacterial infection. Tissue necrosis. High cortisol level.

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6
Q

What is left shift?

A

Due to bacterial infection. There is release of more WBC from bone marrow, but due to more demand there are many immature neutrophils formed. This is called Left shift.

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7
Q

What is marker of FC receptor?

A

CD16

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8
Q

Why is it FC receptordecreased?

A

FC receptor decreased when there are Immature neutrophils.

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9
Q

When does monocytosis occur?

A

In chronic inflammation and malignancy.

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10
Q

What is eosinophilia? When does it occur?

A

Increase in numbers of eosinophils in circulation. It occurs in allergic reactions, parasitic infection and Hodgkin’s lymphoma.

eosinophils increase due to increase IL-5 production

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11
Q

Which is the only bacteria to cause increased WBC count?

A

Bordetella pertussis

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12
Q

What is infectious mononucleosis?

A

it is an EBV infection that results in lymphocytic leukocytosis comprised of CD8 positive T cells.

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13
Q

What will be the response of see T8 positive T cells when EBV infection occurs?

A

It will cause generalized lymphadenopathy and splenomegaly.

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14
Q

Which area of lymph node will be enlarged in an EBV infection of a CD8 positive T cell response?

A

Paracortex. Because T cell live here, so when there will be hyperplasia they will enlarge

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15
Q

Why does splenomegaly occur in CD8 positive t cellsinfection?

A

The white pulp area the spleen has lymphocytes, it has B cell area and T cell area. In this particular situation T cell enlarge making spleen large.

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16
Q

Where will be Expansion in spleen in CD8 positive T cell response?

A

periarteriallymphatic sheath

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17
Q

What do we use for screening of EBV virus?

A

We do monospot test. The test comes positive after one week of infection. A negative test suggests cytomegalovirus. A confirmed diagnosis can be made by EBV viral capsid antigen.

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18
Q

What is acute leukemia?

A

Acute leukemia is defined as accumulation of more than 20% blast cells in bone marrow. In Leukemia., Myeloblast or Lymphoblast loses the ability to make more mature cells. The immature results start to pile up

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19
Q

How did the patient of acute leukemia presents?

A

Patient of acute leukemia present With anemia s\s of hypoxia.

Thrombocytopenia with s\s of bleeding

Infection.

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20
Q

How do we know if the accumulation of blast is of myleoblast or lymphoblast?

A

By certain markers. TDT positive in nucleolus. Is lymphoid accumulation

Myleoperoxide in cytoplasm of blast is myleoidblast accumulation.

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21
Q

Which accumulation is most common in down syndrome after age 5?

A

Lymphoid accumulation. Also known as ALL

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22
Q

What is b-ALL?

A

It is a type of lymphoid Accumulation. It expresses markers from CD 1019 and 20. It has excellent response to chemotherapy. We need to give direct chemotherapy to scrotum and CSF.

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23
Q

What is T-ALL?

A

It is a type of lymphoid accumulation, expresses markers from CD2 to CD8. It does not express CD10. It represents as a thymic mass in teenager in mediastinal thus called acute lymphoblastic lymphoma.

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24
Q

What is AML?

A

It is myleoidblast accumulation It is characterized by staining MPO. It has auer rods in blastic cells. It appears in Old adult 50 to 60 age.

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25
Q

What are the types of acute leukemia?

A

Acute monocytic leukemia. It is proliferation of monoblast and lacks MPO. It’s blasts infiltrate gums.

Acute megakaryoblastic leukemia. Associated with Down syndrome before age 5.

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26
Q

What is myledysplastic syndrome?

A

It occurs in patients who are already exposed to alkylating agents or chemotherapy or radiotherapy.

Due to lack of cells(Cytopenia.) With hypercellular bone marrow(Because cells are trap in there) Most patients die from infection or bleeding.

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27
Q

What is chronic leukemia?

A

Chronic leukemia is neoplastic proliferation of mature circulating lymphocytes.

28
Q

What is chronic lymphocytic leukemia?

A

It is neoplastic. Proliferation of naive B cells. It go expresses CD5 and CD20. There is increase in lymphocytes and smudge cells

29
Q

What is hairy cell leukemia?

A

A chronic leukemia. It is neoplastic qualification of mature B cells. I just connect arise by having Hairy cytoplasmic processes. These cells are positive for TRAP.(Tartrate resistant acis phosphate)

Clinical features include spinal magley due to red pulp. They lack lymphoadenopathy. And they have dried tap with owner respiration because bones are fibrosed.

They gave excellent response to 2-CDA cells.

30
Q

What is ATLL?

A

Adult T cell leukemia lymphoma. Due to proliferation of CD4 positive Tcells.

Its clinical features include rash, generalized lymphadenopathy with hepatosplenomegaly. It has lytic bone lesions with hypercalcemia.

31
Q

what is mycosis fungoides?

A

it is neoplastic proliferation of mature cd positive T cells it’s cells infilterate skin causing rash, plaques and nodules

PAUTRIER MICROABCESSES IN EPIDERMIS OF T CELLS PRESENT

32
Q

What is sezary syndrome?

A

Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma
CLASSIC FINDING CEREBRIFORM NUCLEI.

33
Q

What is Myeloproliferative disorder?

A

The accumulation of mature myeloid cell lineages. For example, RBCS, neutrophil, basophil, eosinophils and megakaryotes.

34
Q

What is chronic myeloid leukemia?

A

The neoplastic proliferation of mature myeloid cells, especially granulocytes. It’s called chronic myeloid leukemia.

35
Q

What is the characteristic Finding in chronic myeloid leukemia?

A

basophils increased

36
Q

What is the first line treatment of chronic myeloid leukemia?

A

The first line treatment is imatinib. It basically blocks tyrosine kinase activity. That is responsible for the overproduction of neoplastic cells.

37
Q

Phases of chronic myeloid leukemia?

A

Chronic Myeloid The spleen enlarges.

Accelerated phase. Enlarging spleen.

Transformation phase. Acute leukemia.

38
Q

In transformation phase, how does chronic myeloid can be transformed in ALL?

A

It is due to the mutation. It occurs at myeloid stage or on the very top hemopoietic stem cell Stage. That is when the transformation occurs, it can either pile up on myleoid causing AML or at lymphoid causing ALL.

39
Q

On blood smear of chronic myeloid leukemia, we see granulocytes are increased, but granulocytes can also be increased in an infection that is leukemoid reaction. How do we know it is CML or leukemoid reaction?

A

1- CML granulocytes are leukocyte alkaline phosphate negative. Leukemoid reaction are positive.

2- Basophils are increased in CML.

3- Basophil short translocation. CML granulocytes exhibit t(9:22).

40
Q

What is polycythemia Vera?

A

t Is neoplastic proliferation of neoplastic cells, especially red blood cells. Its clinical symptoms involve blurry vision and headache due to thickness of blood. There is an increase in risk of venous thrombosis, especially Bud-chiari syndrome. Itching after bathing.

Phlebotomy is treatment

41
Q

How does Polycythemia Vera differentiate from reactive polycythemia?

A

In polycythemia Vera, serum oxygen level is normal and erythropoietin is decreased due to increase in red blood cell by negative feedback. But in reactive polycythemia, due to lung disease, serum oxygen level is decreased in erythropoietin is increased. In renal cell carcinoma There is reactive polycythemia due to erythropoietin production.

42
Q

essential thrombocythemia?

A

The neoplastic proliferation of cells especially platelets.

There is rarely any Fibrosis or acute Leukemia In essential thrombocythemia.

On blood smear, platelets are increased. It can be essential thrombocythemia or Iron deficiency anemia.

43
Q

Which of the Myeloproliferative disorders are associated with JAK2t kinase mutation?

A

Polycythemia Vera.

Essential thrombocythemia.

Myelofibrosis.

44
Q

What is Myelofibrosis?

A

The neoplastic Proliferation of Megakaryocytes. It results in marrow fibrosis.

Its clinical feature includes Splenomegaly.

Leukoerythroblastic smear.

Increased risk of infection, thrombosis and bleeding.

45
Q

in which Myeloproliferative disorder are teardrop red blood cells?

A

myelofibrosis

46
Q

Which region of lymph node is enlarged in reumatoid arthritis?

A

Follicle enlarge

47
Q

When is the paracortex of lymph node enlarged.

A

viral infection like

Infectious mononucleosis

48
Q

What is the lymphoma?

A

The neoplastic proliferation of lymphoid cells that forms mass.

49
Q

What are small new plastic b cell lymphomas?

A

follicular cell lymphoma, mantle cell lymphoma, marginal zone.

50
Q

What is follicular lymphoma?

A

Neoplastic small B cell. That make follicle like nodules. Due to 14;18 BCL 2 translocations

Asymptomatic

Symptomatic Patients should be given rituximab. Patient presents with enlarging lymph node.

51
Q

What is the difference between follicular lymphoma and follicular hyperplasia?

A

In follicular lymphoma. There will be disruption of normal lymph node architecture.

Lack of tangible body macrophages in germinal center. They are only present in follicular hyperplasia.

Expression of BCL 2 in follicles. Not present in follicular hyperplasia.

52
Q

What is mantle cell lymphoma?

A

In mantle cell lymphoma, there will be a neoplastic proliferation of cells. Immediately adjacent to follicular, part of lymph node.

It is due to translocation 11:14 chromosome.

The neoplastic proliferation will be due to because cycling D1 on chromosome 11, translocates to IG heavy chain Locus on chromosome 14. the overexpression of cyclin. D1 promotes G1\ S transition in cell cycle. And due to this continuous transition, there will be the A neoplastic mass called mantle cell Lymphoma.

53
Q

What is marginal cell lymphoma?

A

The neoplastic proliferation of small B cells CD 20 plus cells that expand the marginal zone. It is associated with Hashimoto’s Thyroiditis, sjogren syndrome, (H pylori gastritis. called Maltoma Means marginal zone lymphoma in mucosal sites.)

54
Q

what is Burkitt’s lymphoma.

A

The intermediate sized B cells neoplastic proliferation. In African form, it involves jaw. In sporadic it involes abdomen.

High mitotic rate and stary sky appearance on Histology.

55
Q

Hodgkin’s lymphoma.

A

Neoplastic proliferation of reed Sternberg cells.

large B cells with multiload nuclei and prominent nucleoli.

56
Q

what are subtypes of Hodgkin’s lymphoma.

A

1-Nodular sclerosis: Enlarging neck, lymph node or mediastinal lymph node, any young adult, usually female biopsy shows reed Sternberg cells In. Lake like spaces called lacunar cells present. Lymph nodes are divided by bands of fibrosis.

2-lymphocyte rich.

3- mixed cellularity. Associated with Abundant eosinophils IL-5.

4- lymphocyte depleted.

57
Q

multiple myeloma?

A

malignant proliferation of plasma cells in bone marrow.

58
Q

multiple myeloma?

A

malignant proliferation of plasma cells of bone marrow.

59
Q

clinical findings of multiple myeloma

A

plasma cells over produce various substances which give clinical findings accordingly.
lytic punched out lesions.
M-Spike on electroproresis due to over production of immunoglobins.
high risk of infection due to monoclonal antibodies.
rouleaux formation on blood smear.
primary amyloidosis
Bence-jones proteinuria.

60
Q

MGUS?

monoclonal gammopathy of undetermined significance

A

high serum with M spike

61
Q

Waldenstrom macroglobulinemia

A

B cell lymphoma with monoclonal igM production
generalized LAD with M-spike but no lytic bone lesion
retinal hemorrhage and stroke due to high viscosity of blood
treated by plasmapheresis

62
Q

in which disease there are characterstic birbeck granules ( tennis racket)?

A

langerhans cells histiocytosis

63
Q

letterer-siwe disease

A

malignant
infant less than 2 yrs
skin rash and cystic skeletal defects
rapidly fatal

64
Q

eosinophilic granuloma

A

benign proliferation of langerhans cells in bone

CLASSIC PRESENTATION PATHOLOGIC FRACTURE

65
Q

Hand schuller christian disease

A

malignant proliferation

scalp rash,lytic skulldefects,diabetes insipidus

PATHOMA (4 decks)

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