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Flashcards in WBC DISORDERS- 2 Deck (45):
1

What is the next line of investigation once serum protein electrophoresis shows the presence of a monclonal M protein?

Immunofixation

2

Identify the stage of Hodgkin's Lymphoma described below:

Involvement of a single lymph node region

Stage I

3

 

List 3 chronic inflammatory/autoimmune disorders  from which extranodal marginal zone lymphomas arise. 

Helicobacter gastritis, Hashimoto thyroiditis, Sjogren Syndrome

4

Clinical features of multiple myeloma

Hint= CRAB 

Calcium (elevated) – hypercalcemia

R = Renal failure

A = Anemia

B = Bone lesions (bone pain)

5

Hematologic signs of splenectomy

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nucleated RBCs (NRBCs).

Howell-Jolly (HJ) bodies

target cells (excess membrane cannot be removed

Thrombocytosis- Platelets that would have been normally sequestered in the spleen are now circulating

6

Identify the stage of Hodgkin's Lymphoma described below:

Involvement of lymph node regions on both sides of the diaphragm

Stage III

7

Identify the stage of Hodgkin's Lymphoma described below:

Multiple lymph nodes on both sides of the diaphragm + skletal, pleural and spleen involvement

Stage IV

8

Main components of Hodgkin's Lymphoma

Components

a. neoplastic Reed-Sternberg cells <1% of the overall tumor mass.

b. exuberant tissue response consisting of reactive lymphocytes, granulocytes, macrophages, and plasma cells >90% tumor cellularity

9

affects predominantly children

Multiple cutaneous lesions

round to oval, red-brown, nonscaling papules and small plaques

1. Special stain to identify the main cell in this lesion?

2. Immunohistochemical stain ?

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The diagnosis is  urticaria pigmentosa

1. toluidine blue and Giemsa stains

2. mast cell tryptase and KIT.

10

List 4 pathophysiologic factors implicated in multiple myeloma

A. Pathogenic antibodies or antibody fragments

B. Bone resorption

C. Renal failure

D. Suppression of humoral immunity

11

 

generalized lymphade­nopathy, weight loss, fever

eosinophilia, pruritus

Lymph node biopsy: pleomorphic mixture of variably sized malignant lymphocytes in a background of eosinophils and macrophages

brisk neoangiogenesis

CD2+ CD3+ CD5+

Peripheral T-Cell Lymphoma

12

Identify the stage of Hodgkin's Lymphoma described below:

Two or more lymph node regions on the same side of the diaphragm

Stage II

13

List 4 causes for massive splenomegaly

Primary myelofibrosis

Hairy cell Leukemia

Leishmaniasis

Malaria

14

What conditions are thymomas  associated with?

Myasthenia gravis

15

WHat do the Howel Jolly bodies contain?

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nuclear remnants

16

generalized lymphadenopathy, weight loss, fever 

Lymph node biopsy: diffuse effacement

 large lymphocytes  that exhibit large nuclei, open chromatin, and prominent nucleoli.

high mitotic rate

CD 19+ CD20+ CD10+ BCL-6+

 

Diffuse large B-cell lymphoma (DLBCL)

17

Pruritus and flushing

Rhinorrhea

Darier sign positive

Dermatographism present

 

1. What enzyme levels would you do to confirm diagnosis?

2. Diagnosis?

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1. Serum tryptase- Increased

2. Systemic mastocytosis

18

List the 4 subtypes of Classic Hodgkin's Lymphoma

 

1. Nodular sclerosis

2. Mixed cellularity

3.Lymphocyte-rich

4. Lymphocyte depletion

19

70 year old male presenting with excessive thirst, nausea, constipation, loss of appetite, and confusion. ESR 95mm/1st hour

PS shows rouleaux formation.

Skull Xray shows attached findings

1. What are you likely to see on a BM aspirate?

2. List 2 immunophenotypical markers for this condition?

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1.  plasmablasts with vesicular nuclear chromatin and a prominent single nucleolus; bizarre, multinucleated cells; normal plasma cells

2. CD138, CD56

20

70 year old male

Generalized lymphadenopathy

Splenomegaly

absolute lymphocyte count > 5000 per mm3

Peripheral smear: Image attached

CD19+,CD20+, CD 23+ and CD5+

1. What are the 2 forms of presentation of this condition?

2. This condition is assoc with increased incidence of which anemia?

 

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1. Chronic lymphocytic leukemia OR small lymphocytic lymphoma

2. autoimmune hemolytic anemia (AIHA) 

21

1. Diagnosis?

serum M protein level is less than 3 gm/dL

Asymptomatic

2. What is the rate of transformation to Myeloma ?

1. Monoclonal Gammopathy of Uncertain Significance

2. 1% per year

22

solitary lesion of bone or soft tissue

Bone- spine, ribs etc i.e. axial skeleton

Soft tissue- lungs, oronasopharynx, or nasal sinuses

Modest elevations of M proteins 

Solitary Myeloma (Plasmacytoma).

23

Clinicopathologic presentation of Langerhan cell histiocytosis

  1. Letterer-Siwe disease
  2. eosinophilic granuloma
  3. Hand-Schüller-Christian triad
  4. Pulmonary Langerhans cell histiocytosis

24

Type of amyloidosis likely to be seen in amyloidosis resulting from renal failure in multiple myeloma?

AL (k) type

25

List the criteria for hypersplenism

Hint: Hyper Splenism Ravages Cells

Hypercellular or normal marrow

Splenomegaly

Response to splenectomy

Cytopenias

26

1. List 3 consequences of bone resorption in multiple myeloma

 

2. List 3 causes for renal failure in Mutliple myeloma

3. The important cytokine required for survival and proliferation of multiple myeloma cells

1. pathologic fractures, bone pain, and hypercalcemia

2. precipitation of free light chains+pyelonephritis+ hypercalcemia

3. IL-6

27

Identify the cell described below

Large cell, multiple nuclei or a single nucleus with multiple nuclear lobes, each with a large inclusion-like nucleolus about the size of a small lymphocyte, abundant cytoplasm

Reed-Sternberg cell

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28

Splenic dysfunction would predispose to infections by which particular  type of organisms

encapsulated pathogens- pneumococci, meningococci, and Haemophilus influenzae, Salmonella

29

generalized pruritic rash 

Skin biopsy image attached

epidermis and upper dermis are infiltrated by PAS

positive cells 

CLA+ CCR4+CCR10+

1. What is the name given to the aggregates of atypical lymphoctes within the epidermis?

2. Diagnosis?

Q image thumb

1. Pautrier microabscesses

2. Mycosis Fungoides

30

70 year old male

Generalized lymphadenopathy

Splenomegaly

Lymph node biopsy: monotonous collection of small, round lymphocytes , scant cytoplasm, condensed chromatin mixed with prolymphocytes

Immunophenotype: CD19+,CD20+, CD 23+ and CD5+

This hematologic condition is assoc with a risk of transformation to which type?

Transformation to diffuse large B-cell lymphoma - Richter syndrome

31

See the attached image and interpret

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two observations:

a. üsharp band in the patient serum is cross-linked by antisera specific for IgG heavy chain (G) and kappa light chain (κ), indicating the presence of an IgGκ M protein

b. normal immunoglobulins are suppressed

32

painless, generalized lymphadenopathy

Lymph node biopsy: replacement of normal architecture by neoplastic follicles that mimic the appearance of normal germinal centers, composed of centrocytes and centroblasts

t(14;18)

Immunophenotyping: CD19, CD20, CD10, surface Ig, BCL 2 and BCL6 positive

1. What is the consequence of the translocation?

2. Diagnosis?

3. Risk of transformation to which type?

 

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1. overexpression of BCL2

2. Follicular lymphoma

3. 30-50% transform to - diffuse large B-cell lymphoma

33

child

mandibular mass

subequatorial Africa

Biopsy: diffuse infiltrate of intermediate-sized lymphoid cells interspersed with nuclear remnants of apoptotic cells phagocytosed by macrophages

surface IgM+ CD19+ CD20+ CD10+  BCL6+  BCL 2-

What is the translocation assoc with this condition?

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t(8;14)-translocations of the MYC gene on chromosome 8 lead to increased MYC

34

70 year old male presenting with excessive thirst, nausea, constipation, loss of appetite, and confusion. ESR 95mm/1st hour

PS shows rouleaux formation.

BM aspirate shows findings as seen in the attached image.

1. What is the skull xray likely to show?

2. What is the serum/urine electrophoresis likely to show?

3. What type of protein is excreted in the urine?

Q image thumb

1. punched-out defects, 1- 4 cm in diameter

2. abnormal protein “spikes” in serum or urine electrophoresis- M protein

3. Free light chains aka Bence Jones proteins

35

middle aged male

massive splenomegaly

Peripheral smear : round, oblong, or reniform nuclei and moderate amounts of pale blue cytoplasm with threadlike or bleblike extensions

BM aspirate: dry tap

Immunophenotype: CD19 CD20, surface IgG,

CD11c+ CD25+ CD103+ annexin A1+

What stain do these cells stain positive for?

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TRAP (tartrate-resistant acid phosphatase) stain

36

Components of DiGeorge Syndrome

 

Cardiac defects

Abnormal facies

Thymic hypoplasia - cellular immune deficiency: abnormal number and function of T-cells)

Cleft palate

Hypoparathyroid with hypocalcemia - tetany

22 chrom defect deletion 22q11

37

2 forms of localized mastocytosis

urticaria pigmentosa

solitary mastocytoma

38

1.What does the M component in Multiple myeloma represent?

2. List two pathogenic effects of light chains

1. monoclonal Ig - usually IgG or IgA type

2. The toxic effect of light chains are as follows:

a. toxic to renal epithelial cells  - form precipitates and obstructive casts

b. amyloid, fibrillar deposits in  renal glomeruli perivascular spaces of liver, spleen, and heart

39

Identify the lesion whose light and electron microsocpic features have been depicted in the image.

What are the IHC markers that you would use to confirm your diagnosis?

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1.Langerhans Cell Histiocytosis

2. HLA-DR, S-100, and CD1a

40

Japan, West Africa, and the Caribbean basin

generalized lymphadenopathy

hepatosplenomegaly

peripheral blood lymphocytosis

hypercalcemia

1. What is the role of the protein encoded by the causative Virus?

2. Diagnosis?

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1. Tax protein - potent activator of NF-κB, enhances lymphocyte growth and survival.

2. Adult T-Cell Leukemia/Lymphoma

The causative virus is human T-cell leukemia retrovirus type 1 (HTLV-1)

41

generalized exfoliative erythroderma + peripheral smear filled with cells shown in the attached image

Cells are PAS positive

Immunophenotype: CLA+ CCR4+ and CCR10+

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Sézary Syndrome

42

painless lymphadenopathy

splenic mass

Biopsy: homogeneous population of small lymphocytes, irregular to occasionally deeply clefted (cleaved) nuclear contours, condensed chromatin, inconspicuous nucleoli, scant cytoplasm

Immunophenotype: 

CD 23- Cyclin D1+ CD 19+  CD 20+

What's the translocation assoc with this lymphoma?

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t(11;14) -overexpression of cyclin D1

43

List atleast 1 example of each category of causes of splenomegaly

a. Infectious

b. Congestive States Related to Portal Hypertension

c. Lymphohematogenous Disorders

d. Storage Diseases

e. Immunologic-Inflammatory Conditions

Infections- Typhoid fever, IM, Malaria, Leishmaniasis

Congestive States Related to Portal Hypertension- Liver cirrhosis, cardiac decompensation of right side.

Lymphohematogenous Disorders – Myeloproliferative neoplasms

Storage Diseases- Gaucher, Nieman Pick, Mucopolysaccharidoses

Immunologic-Inflammatory Conditions- Rheumatoid arthritis, SLE

44

young adult

soft tissue mass

large anaplastic cells, horseshoe-shaped nuclei and voluminous cytoplasm

CD 30 positive

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Anaplastic Large-Cell Lymphoma

45

Identify the lymphoma described below:

  1. deposition of collagen in bands that divide involved lymph nodes into circumscribed nodules
  2. polymorphous background of T cells, eosinophils, plasma cells, and macrophages
  3. positive for  CD15, and CD30, negative for other B-cell markers, T-cell markers, and CD45 (leukocyte common antigen)

 

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Nodular Sclerosis- most common form of HL

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