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Diseases (LHS) > White Blood Cell Disorders > Flashcards

White Blood Cell Disorders Flashcards

1
Q

Generally speaking, how can you distinguish NHL from HL?

A

NHL appears with HS that involves the entire node and that has cells that are all malignant, while HL will present with Reed Stemberg cells surrounded by tons of lymphocytes.

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2
Q

What CD are NHL B-cells positive For?

A

CD20

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3
Q

What are the low grade NHL?

A

CLL/SLL, Mantle cell (t 11,14), Follicular (t 14,18), Marginal (t 11,18), and Lymphoplasmacytoid (t 9,14)

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4
Q

What are the high grade NHL?

A

Diffuse large B cell (t 14,18), and Burkitt’s (t 8,14), (t 2,8), or (t 8,22)

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5
Q

What systems are involved in CLL/SLL?

A

Expect involvement of bone marrow and lymph node. Associated with trisomy 12.

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6
Q

What HS finding for CLL/SLL?

A

Presence of smudge cells.

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7
Q

What occurs in Mantle cell lymphoma?

A

Overproduction of Cyclin D1 (t 11 14)

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8
Q

What occurs in Follicular cell lymphoma?

A

Overproduction of Bcl2 (t 14 18). It is more pale nodular. Associate with markers CD19, CD20, and CD10

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9
Q

What occurs in Marginal cell lymphoma?

A

Associated with Trisomy 18 (t 11,18). History would include h. pylori, hashimoto’s, or sjogrens.

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10
Q

What occurs in lymphoplasmacytoid lymphoma?

A

Cells are produced that are half lymphocyte/half plasma cell (t 9 14). Associated with Waldenstrom’s Macroglobulinemia.

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11
Q

What makes a lymphoma high grade?

A

It proliferates rapidly.

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12
Q

What translocation is diffuse B cell lymphoma? What CD markers does it show?

A

t 14 18

Arises usually from follicular NHL.

CD19, CD20, CD22

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13
Q

What occurs in Burkitt’s lymphoma? What do you expect on HS? What translocations is it confirmed with?

A

C-myc mutation associated usually with EBV

Starry sky appearance on scope

t 8 22, t 8 14, t 2 8

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14
Q

What CD marker is associated with the cells in HL?

A

CD15/30

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15
Q

What two types of HL are there?

A

Classical (Mixed cellularity, nodular sclerosis, lymphocyte depletion, and lymphocyte rich classic HL)
Nodular Lymphocyte Predominant HL

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16
Q

What cells will nodular lymphocyte predominant HL present with?

A

Lacunar cells surrounded by fibrosis/sclerosis

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17
Q

What clinical symptoms are associated with HL?

A

Low grade fever, night sweats, weight loss, mediastinal lymphadenopatjy

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18
Q

What are the four plasma cell disorders?

A

Multiple myeloma, Monoclonal Gammopathy of Undetermined Significance, Waldenstrom Macroglobulinemia, and Langerhan Cell Histiocytes

19
Q

Multiple myeloma is the malignant proliferation of plasma cells that typically occurs in older men. What clinical features do you expect?

A

Bone lesions with hypercalcemia, Rouxleaus formation of RBCs, and primary AL amylooidosis (kappa light chains).

Expect an M spike, but do not confuse with MGUS.

20
Q

What does MGUS show on SPEP?

A

It shows an increased M spike. You will know it from MM, because it will have none of the MM clinical manifestations.

21
Q

Waldenstrom Macroglobulinemia is a B-cell lymphoma with monoclonal IgM production associated with lymphoplasmacytoid NHL. What clinical features do you expect?

A

Neurological defects with an M spike on SPEP.

22
Q

What is the main granule that shows for Langerhan Cell Histiocytes?

A

Birbeck granules!

23
Q

How can you tell a vignette is an eosinophilic granuloma (Langerhan’s Cell Histiocyte?)

A

It will be a single bony lesion, with S100+ and CD19+ markers.

24
Q

How can you tell a vignette is an Hand-Schuler-Christian (Langerhan’s Cell Histiocyte?)

A

Multiple lesions will be present, with diabetes insipidus likely happening in the child.

25
Q

How can you tell a vignette is an Letterer-Siwe (Langerhan’s Cell Histiocyte?)

A

It will be an infant with a skin rash who has multiple lesions and poor prognosis.

26
Q

How can you distinguish myelodysplastic, myeloproliferative, and leukemia conditions?

A

Myelodysplastic is abnormal maturation of cell lines, myeloproliferative is overproduction of cell lines, and leukemia is overproduction of ONE type of cell.

27
Q

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in CML? What is its characteristic issue? How do you treat it?

A

CML = basophils

Driven by Philly chromosome (t 9 22): Bcr-abl

Treatment is GLEEVNEC/imatinib

28
Q

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in PV? What is its characteristic issue? How do you treat it?

A

PV = RBCs

Associated with JAK2 kinase mutation

Complete a phlebotomy for viscous blood, then hydroxyurea. Expect an increased RBC volume, normal arterial oxygen saturation, and splenomegaly with leukocyte ALP >100.

29
Q

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in ET? What is its characteristic issue? How do you treat it?

A

ET = platelets

JAK 2 kinase mutation

Treat with hydroxyurea and low-dose aspirin

30
Q

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in myelofibrosis? What is its characteristic issue? How do you treat it?

A

Myelofibrosis = megakaryocytes

Associated with JAK 2 mutation

Megakaryocytes produce excess PDGF causing marrow fibrosis.

31
Q

You should consider a myelodysplastic disorder if a patient presents with an anemia that has no other explanation. What are the three subtypes? What characteristic finding on HS?

A

Ringed sideroblasts

  1. Refractory anemia with excess blasts (RA-EB)
  2. Refractory anemia with excess blasts in transformation
  3. Chronic myelomonocytic leukemia
32
Q

Acute leukemia is characterized by “blasts,” which are immature WBCs with “punched out” nucleoli. It has two types: ALL and AML. What is ALL?

A

Acute lymphoblastic leukemia

33
Q

What is characteristic of ALL?

A

Presence of TdT, a mediastinal mass in a child is probably T-cell ALL

34
Q

What types of ALL are there?

A

B-cell (more common, but plague adults leading to poor prognosis)

T-cell (more common in kids and has good prognosis)

35
Q

Acute leukemia is characterized by “blasts,” which are immature WBCs with “punched out” nucleoli. It is also associated with Auer rods and granules in cytoplasm. It has two types: ALL and AML. What is AML?

A

Acute myeloid leukemia

36
Q

What population is AML most common?

A

Older adults

37
Q

What is characteristic of a type of AML, APL?

A

Auer rods on HS with a t 15 17 translocation of retinoic acid receptor. Treat with all trans retinoic acid.

38
Q

What is characteristic of a type of AML, acute monocytic leukemia?

A

Proliferation of monoblasts, usually lack TPO, and blasts are characteristically infiltrating the gums

39
Q

What is characteristic of a type of AML, acute megakaryoblastic leukemia?

A

Associated with Down syndrome.

40
Q

From what condition can AML arise?

A

Myelodysplastic disorders (i.e. RA-EB, etc)

41
Q

Chronic leukemias include CLL, hairy cell leukemia, and adult T cell leukemia/lymphoma. What is characteristic of CLL?

A

CLL involves proliferation of naive B cells that co-express CD5 and CD20. It is the most common leukemia overall.

Will see “smudge” cells on HS

Treat with rituxamab.

Often leads to SLL

42
Q

Chronic leukemias include CLL, hairy cell leukemia, and adult T cell leukemia/lymphoma. What is characteristic of hairy cell leukemia?

A

Occurs in middle aged men usually. Involves proliferation of mature B cells characterized by hairy cell processes.

Cells are TRAP positive.

Usually “dry tap” on aspirate

Treat with 2-CDA cladribine

43
Q

Chronic leukemias include CLL, hairy cell leukemia, and adult T cell leukemia/lymphoma. What is characteristic of adult T cell leukemia/lymphoma?

A

Proliferation of CD4+ T cells. Associated with HTLV-1 infection.

Diseases (LHS) (3 decks)

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