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PATHPROS > WBC DISORDERS- 2 > Flashcards

WBC DISORDERS- 2 Flashcards

Lymphomas Disorders of spleen and thymus

1
Q

Identify the stage of Hodgkin’s Lymphoma described below:

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

Deborah Dalmeida MD

A

Stage II

Deborah Dalmeida MD

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2
Q

Identify the stage of Hodgkin’s Lymphoma described below:

Involvement of lymph node regions on both sides of the diaphragm

Deborah Dalmeida MD

A

Stage III

Deborah Dalmeida MD

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3
Q

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

Cells are PAS positive

Immunophenotype: CLA+ CCR4+ and CCR10+

Deborah Dalmeida MD

A

Sézary Syndrome

Deborah Dalmeida MD

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4
Q

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?

Deborah Dalmeida MD

A
  1. Langerhans Cell Histiocytosis
  2. HLA-DR, S-100, and CD1a

Deborah Dalmeida MD

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5
Q

young adult

soft tissue mass

large anaplastic cells, horseshoe-shaped nuclei and voluminous cytoplasm

CD 30 positive

Deborah Dalmeida MD

A

Anaplastic Large-Cell Lymphoma

Deborah Dalmeida MD

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6
Q

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)

Deborah Dalmeida MD

A

Nodular Sclerosis- most common form of HL

Deborah Dalmeida MD

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7
Q

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?

Deborah Dalmeida MD

A

Transformation to diffuse large B-cell lymphoma - Richter syndrome

Deborah Dalmeida MD

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8
Q

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?

Deborah Dalmeida MD

A

t(11;14) -overexpression of cyclin D1

Deborah Dalmeida MD

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9
Q

Main components of Hodgkin’s Lymphoma

Deborah Dalmeida MD

A

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

Deborah Dalmeida MD

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10
Q

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?

Deborah Dalmeida MD

A
  1. overexpression of BCL2
  2. Follicular lymphoma
  3. 30-50% transform to - diffuse large B-cell lymphoma

Deborah Dalmeida MD

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11
Q

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

Deborah Dalmeida MD

A

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

Deborah Dalmeida MD

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12
Q

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

Deborah Dalmeida MD

A

Helicobacter gastritis, Hashimoto thyroiditis, Sjogren Syndrome

Deborah Dalmeida MD

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13
Q

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 ?

Deborah Dalmeida MD

A

The diagnosis is urticaria pigmentosa

  1. toluidine blue and Giemsa stains
  2. mast cell tryptase and KIT.

Deborah Dalmeida MD

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14
Q

2 forms of localized mastocytosis

Deborah Dalmeida MD

A

urticaria pigmentosa

solitary mastocytoma

Deborah Dalmeida MD

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15
Q

Clinical features of multiple myeloma

Hint= CRAB

Deborah Dalmeida MD

A

Calcium (elevated) – hypercalcemia

R = Renal failure

A = Anemia

B = Bone lesions (bone pain)

Deborah Dalmeida MD

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16
Q

List the 4 subtypes of Classic Hodgkin’s Lymphoma

Deborah Dalmeida MD

A
  1. Nodular sclerosis
  2. Mixed cellularity
  3. Lymphocyte-rich
  4. Lymphocyte depletion

Deborah Dalmeida MD

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17
Q

Laboratory indicators of tumpr lysis syndrome

Deborah Dalmeida MD

A

Atleast 2 of the following:

Hyperuricemia

Hyperkalemia

Hyperphosphatemia

Hypocalcemia

Deborah Dalmeida MD

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18
Q

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+

Deborah Dalmeida MD

A

Diffuse large B-cell lymphoma (DLBCL)

Deborah Dalmeida MD

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19
Q

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?

Deborah Dalmeida MD

A

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

Deborah Dalmeida MD

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20
Q

List 4 causes for massive splenomegaly

Deborah Dalmeida MD

A

Primary myelofibrosis

Hairy cell Leukemia

Leishmaniasis

Malaria

Deborah Dalmeida MD

21
Q
  1. One important clinical feature of tumor lysis syndrome
  2. How do you prevent tumor lysis syndrome?

Deborah Dalmeida MD

A
  1. Renal failure - due to crystal-induced tissue injury
  2. Aggressive hydration, allopurinol, rasburicase

Deborah Dalmeida MD

22
Q

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

Deborah Dalmeida MD

A

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

Deborah Dalmeida MD

23
Q

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?

Deborah Dalmeida MD

A
  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

Deborah Dalmeida MD

24
Q

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

Deborah Dalmeida MD

A

Immunofixation

Deborah Dalmeida MD

25
Q

Identify the stage of Hodgkin’s Lymphoma described below:

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

Deborah Dalmeida MD

A

Stage IV

Deborah Dalmeida MD

26
Q

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

Deborah Dalmeida MD

A

Solitary Myeloma (Plasmacytoma).

Deborah Dalmeida MD

27
Q

Pruritus and flushing

Rhinorrhea

Darier sign positive

Dermatographism present

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

Deborah Dalmeida MD

A
  1. Serum tryptase- Increased
  2. Systemic mastocytosis

Deborah Dalmeida MD

28
Q

WHat do the Howel Jolly bodies contain?

Deborah Dalmeida MD

A

nuclear remnants

Deborah Dalmeida MD

29
Q
  1. What does the M component in Multiple myeloma represent?
  2. List two pathogenic effects of light chains

Deborah Dalmeida MD

A
  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

Deborah Dalmeida MD

30
Q

List 4 important events resulting in the finding depicted in the image

Deborah Dalmeida MD

A

a. cytokine storm
b. massive elevation of ferritin and c. sCD25
c. widespread macrophage activation

Deborah Dalmeida MD

31
Q

1. Diagnosis?

serum M protein level is less than 3 gm/dL

Asymptomatic

2. What is the rate of transformation to Myeloma ?

Deborah Dalmeida MD

A
  1. Monoclonal Gammopathy of Uncertain Significance
  2. 1% per year

Deborah Dalmeida MD

32
Q

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?

Deborah Dalmeida MD

A
  1. Pautrier microabscesses
  2. Mycosis Fungoides

Deborah Dalmeida MD

33
Q

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

Deborah Dalmeida MD

A

AL (k) type

Deborah Dalmeida MD

34
Q

See the attached image and interpret

Deborah Dalmeida MD

A

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

Deborah Dalmeida MD

35
Q
  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

Deborah Dalmeida MD

A
  1. pathologic fractures, bone pain, and hypercalcemia
  2. precipitation of free light chains+pyelonephritis+ hypercalcemia
  3. IL-6

Deborah Dalmeida MD

36
Q

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

Deborah Dalmeida MD

A

Reed-Sternberg cell

Deborah Dalmeida MD

37
Q

Clinicopathologic presentation of Langerhan cell histiocytosis

Deborah Dalmeida MD

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

Deborah Dalmeida MD

38
Q

List the criteria for hypersplenism

Hint: Hyper Splenism Ravages Cells

Deborah Dalmeida MD

A

Hypercellular or normal marrow

Splenomegaly

Response to splenectomy

Cytopenias

Deborah Dalmeida MD

39
Q

Components of DiGeorge Syndrome

Deborah Dalmeida MD

A

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

Deborah Dalmeida MD

40
Q

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+

Deborah Dalmeida MD

A

Peripheral T-Cell Lymphoma

Deborah Dalmeida MD

41
Q

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?

Deborah Dalmeida MD

A

TRAP (tartrate-resistant acid phosphatase) stain

Deborah Dalmeida MD

42
Q

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?

Deborah Dalmeida MD

A
  1. plasmablasts with vesicular nuclear chromatin and a prominent single nucleolus; bizarre, multinucleated cells; normal plasma cells
  2. CD138, CD56

Deborah Dalmeida MD

43
Q

What conditions are thymomas associated with?

Deborah Dalmeida MD

A

Myasthenia gravis

Deborah Dalmeida MD

44
Q

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?

Deborah Dalmeida MD

A
  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)

Deborah Dalmeida MD

45
Q

Hematologic signs of splenectomy

Deborah Dalmeida MD

A

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

Deborah Dalmeida MD

46
Q

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?

Deborah Dalmeida MD

A
  1. Chronic lymphocytic leukemia OR small lymphocytic lymphoma
  2. autoimmune hemolytic anemia (AIHA)

Deborah Dalmeida MD

47
Q

List 4 pathophysiologic factors implicated in multiple myeloma

Deborah Dalmeida MD

A

A. Pathogenic antibodies or antibody fragments

B. Bone resorption

C. Renal failure

D. Suppression of humoral immunity

Deborah Dalmeida MD

48
Q

Identify the stage of Hodgkin’s Lymphoma described below:

Involvement of a single lymph node region

Deborah Dalmeida MD

A

Stage I

Deborah Dalmeida MD

PATHPROS (36 decks)

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