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Pathology > 5. Diseases of White Blood Cells - BP > Flashcards

5. Diseases of White Blood Cells - BP Flashcards

1
Q

What are the 4 main types of leukemias?

A
  1. Acute lymphoid
  2. Acute myeloid
  3. Chronic lymphoid
  4. Chronic myeloid
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2
Q

What is the clinical picture in patients with de novo 5q- syndrome?

A

Older women with refractory macrocytic anemia + normal or elevated platelet counts.

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3
Q

What are the 4 main microscopic findings in MDS?

A
  1. Macrocytic erythroid cells with basophilic stippling.
  2. Hypogranular and hypolobated neutrophils –> Pseudo Pelger-Huet anomaly).
  3. Micromegakaryocytes
  4. Normocellular/ hypercellular bone marrow
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4
Q

What is the clinical presentation in patients with MDS?

A
  1. Signs and symptoms of cytopenia.

2. 25% have splenomegaly.

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5
Q

What are mainly the microscopic findings in lymphoid leukemias?

A

High nuclear to cytoplasmic ratio - FEW nucleoli.

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6
Q

What are mainly the microscopic findings in myeloid leukemias?

A

More prominent cytoplasm - MORE PROMINENT nucleoli.

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7
Q

What are mainly the microscopic findings in acute leukemias?

A

BLASTS with euchromatin –> SMOOTH nucleus.

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8
Q

What are mainly the microscopic findings in chronic leukemias?

A

MYELOCYTES (more mature) that do NOT using chromatin –> HETEROCHROMATIN –> GRANULAR nucleus.

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9
Q

Is there a male predominance in CLL?

A

YES

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10
Q

What are the markers of CLL?

A

CD19, 20, 23, 5(!)

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11
Q

Mention some associated mutations that we see in CLL.

A
  1. del 13q12-14
  2. Trisomy 12
  3. del 11q
  4. del 17p
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12
Q

What is the prognosis in CLLs associated with a mutation?

A

POOR

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13
Q

Mention some complications of CLL.

A
  1. Hypogammaglobulinemia
  2. 10-15% of patients develop autoimmune hemolytic anemia.
  3. Immune thrombocytopenia
  4. 5% –> DLBCL (Richter transformation)
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14
Q

What are the microscopic findings in CLL?

A
  1. Cells are CRACKED or GINGERSNAP appearance.

2. Smudge cells during preparation.

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15
Q

What is the clinical presentation of CLL?

A

Symptoms –> Most ASYMPTOMATIC…some fatigue and weight loss.
Signs –> Normal physical examination…some splenomegaly.

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16
Q

What is the blast crisis seen in CML?

A

2/3 of CML –> AML
1/3 of CML –> ALL
POOR prognosis.

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17
Q

What is the microscopic morphology of CML?

A
  1. Incr. WBC count –> from myelocytes to band and segmented neutrophils.
  2. BASOPHILS UP!!!
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18
Q

What are the main symptoms of CML?

A
  1. 40% of patients –> ASYMPTOMATIC.
  2. Fatigue + Lethargy + Shortness of breath.
  3. Weight loss + early satiety due to splenomegaly.
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19
Q

What are the lab findings in CML?

A
  1. WBC way up! (median 170.000)
  2. LAP down.
  3. High uric acid.
  4. High lactate dehydrogenase.
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20
Q

What is the clinical course of CML?

A

Indolent phase 3-5 yrs –> Accelerated phase (fever, weight loss, night sweats etc.) –> Blast crisis.

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21
Q

What percentage of patients with HL have constitutional symptoms?

A

1/3

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22
Q

What is the MC presenting symptom in HL patients?

A

Painless cervical lymphadenopathy.

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23
Q

Mention a classic and specific symptom in HL, that is rare.

A

Pain in the lymph nodes with ingestion of alcohol.

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24
Q

What are the markers for HL nodular sclerosis type?

A

CD15, 30 + RSCs are negative for EBV.

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25
Q

Where do we find more easily RSCs? In nodular sclerosis or in mixed cellularity HL type?

A

In mixed cellularity

26
Q

What are the markers for mixed cellularity HL?

A

CD15, 30 + RSCs are positive for EBV.

27
Q

What is the microscopic morphology of lymphocyte predominant HL?

A

Vaguely nodular –> RSCs variants referred to as L&H, or popcorn cells.

28
Q

What are the markers in lymphocyte predominant HL?

A

CD15,30 NEGATIVE + CD20 positive and EMA (epithelial membrane antigen)+.

29
Q

Mention 2 important points about lymphocyte predominant HL.

A
  1. Very benign and often cured with local excision.

2. Often present with cervical or axillary lymphadenopathy.

30
Q

How do we stage in HL?

A

Upon the NUMBER of lymph nodes involved (stage I is 1 lymph node) + the LOCATION of lymph nodes in relation to the DIAPHRAGM.
STAGE II –> One or more lymph nodes on the same side of the diaphragm.
STAGE III –> One or more lymph nodes on opposite sides of the diaphragm.
STAGE IV –> Disseminated disease.

31
Q

Mention an important general point about NHLs.

A

They are often DISSEMINATED at the time of diagnosis –> aggresive NHLs have a propensity for involving LEPTOmeninges.

32
Q

What is the typical clinical presentation of NHLs?

A
  1. Painless lymphadenopathy (MC neck, inguinal, axillary)
  2. 20% of patients have constitutional symptoms –> Fever, weight loss, night sweats- “B”symptoms –> common in aggresive NHLs.
33
Q

What is the main difference between indolent (follicular, SLL) and aggresive (Burkitt, DLBCL) NHLs?

A

The aggressive are more responsive to chemo, hence they can be cured.

34
Q

What is the worst NHL?

A

Mantle cell lymphoma.

35
Q

What is the OCCASIONAL cytogenetic abnormality in follicular lymphoma?

A

Translocation involving bcl-6 on 3q27, and not the usual bcl-2 gene on 18.

36
Q

What are the markers for follicular lymphoma?

A

CD19, 20, 10, surface Ig, bcl-2.

37
Q

What is the prognosis for follicular lymphoma patients?

A

7-9 years –> MOST progress to DLBCL or Burkitt.

38
Q

Is bone marrow involvement frequent in follicular lymphoma?

A

Yes –> 85% of cases.

39
Q

What is the typical clinical presentation of follicular lymphoma?

A
  1. Painless lymphadenopathy
  2. Fever, night sweats, fatigue
  3. MOST patients (80-90%) present with stage III or IV.
40
Q

What is the microscopic morphology of SLL?

A

Proliferation centers - clusters of mitotically active prolymphocytes.

41
Q

What is the main difference between SLL and CLL?

A

They are essentially the SAME process.
SLL–> More prominent the involvement of the lymph nodes.
CLL–> More prominent the involvement of the bone marrow and blood.

42
Q

What is the cytogenetic abnormality in DLBCL?

A

10-20% have the t(14;18) –> bcl-2

30% have a translocation involving the bcl-6 on 3q27.

43
Q

What are the markers of DLBCL?

A

CD19, 20, 79a, surface Ig.

44
Q

What is the microscopic morphology of DLBCL?

A

Monotonous sheets of large cells with prominent nucleoli.

45
Q

Mention 2 important points regarding DLBCL.

A
  1. Some cases of DLBCL –> EBV association (in HIV and immunosuppressed patients).
  2. Primary effusion lymphoma in body cavities –> HHV-8 association.
46
Q

Besides the classic t(8;14), mention 2 other cytogenetic abnormalities seen in Burkitt lymphoma.

A

MYC translocated adjacent to Ig light chain gene :

  1. κ on 2 –> t(2;8)
  2. λ on 22 –> t(8;22)
47
Q

What are the markers for Burkitt lymphoma?

A

CD10, 19, 20, bcl-6, surface Ig.

48
Q

Mention one important fact about Burkitt lymphoma.

A

Has the highest turnover rate of ANY human malignancy –> extremely high risk of tumor lysis syndrome.

49
Q

What is the target group of precursor T cell lymphoblastic leukemia/lymphoma?

A

Adolescents –> aged 15-20.

50
Q

What are the markers for precursor T cell lymphoblastic leukemia/lymphoma?

A

TdT, CD2, 7.

51
Q

What is the gross morphology of precursor T cell lymphoblastic leukemia/lymphoma?

A

Usually appears as a mediastinal mass.

52
Q

What is the microscopic morphology of precursor T cell lymphoblastic leukemia/lymphoma?

A

Lymphoblasts with irregular nuclear contours, small nucleoli, and scant cytoplasm.

53
Q

Mention 2 important points regarding precursor T cell lymphoblastic leukemia/lymphoma.

A
  1. Propensity involving the bone marrow.

2. Propensity to relapse and involve the LEPTOmeninges.

54
Q

What are the markers for mantle cell lymphoma?

A

CD19, 20, 5, Ig, positive - NEGATIVE for CD23 + HIGH levels of cyclin D1.

55
Q

Mention 2 important points regarding mantle cell lymphoma.

A
  1. Survival 3-5 yrs –> NO cure.

2. Can involve GI tract –> producing polyps –> lymphomatoid polyposis.

56
Q

What is mycosis fungoides/Sezary syndrome?

A

A chronic T-cell lymphoma with infiltration of the EPIdermis and DERMIS.
Sezary syndrome is a generalized EXFOLIATIVE dermatitis with leukemic spread.

57
Q

What is the mechanism for the osteolytic lesions in multiple myeloma?

A

Production of IL-6 by neoplastic cells stimulates osteoclasts through the RANK ligand.

58
Q

What Ig are produced in multiple myelomas?

A

60% –> IgG
20-25%–> IgA
15-20% –> λ/κ

59
Q

What is the main cause of death in multiple myeloma?

A

Infection (second comes renal failure).

60
Q

What percentage of multiple myeloma patients do NOT have a detectable M spike, but they do have free light chains in the urine?

A

20%

61
Q

What is the microscopic morphology of multiple myeloma?

A
  1. > 30% of marrow –> Plasma cells.
  2. Accumulation of Ig in plasma cells - Russell bodies.
  3. On peripheral blood smear –> rouleaux formation of RBCs.
62
Q

Describe briefly the general condition of leukemia.

A

The neoplastic cells replace the normal hematopoietic population –> patients will have anemia (pallor and fatigue) + thrombocytopenia (bleeding) + leukopenia (infections).

Pathology (16 decks)

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