WBC DISORDERS- 2 Flashcards by Deborah Dalmeida MBBS MD MEHP

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

Stage II

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Identify the stage of Hodgkin’s Lymphoma described below:

Involvement of lymph node regions on both sides of the diaphragm

Deborah Dalmeida MD

Stage III

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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

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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

Langerhans Cell Histiocytosis
HLA-DR, S-100, and CD1a

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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

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Identify the lymphoma described below:

deposition of collagen in bands that divide involved lymph nodes into circumscribed nodules
polymorphous background of T cells, eosinophils, plasma cells, and macrophages
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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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

Transformation to diffuse large B-cell lymphoma - Richter syndrome

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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

t(11;14) -overexpression of cyclin D1

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Main components of Hodgkin’s Lymphoma

Deborah Dalmeida MD

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

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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

overexpression of BCL2
Follicular lymphoma
30-50% transform to - diffuse large B-cell lymphoma

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

Deborah Dalmeida MD

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

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

Deborah Dalmeida MD

Helicobacter gastritis, Hashimoto thyroiditis, Sjogren Syndrome

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affects predominantly children

Multiple cutaneous lesions

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

Special stain to identify the main cell in this lesion?
Immunohistochemical stain ?

Deborah Dalmeida MD

The diagnosis is urticaria pigmentosa

toluidine blue and Giemsa stains
mast cell tryptase and KIT.

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2 forms of localized mastocytosis

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urticaria pigmentosa

solitary mastocytoma

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Clinical features of multiple myeloma

Hint= CRAB

Deborah Dalmeida MD

Calcium (elevated) – hypercalcemia

R = Renal failure

A = Anemia

B = Bone lesions (bone pain)

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List the 4 subtypes of Classic Hodgkin’s Lymphoma

Deborah Dalmeida MD

Nodular sclerosis
Mixed cellularity
Lymphocyte-rich
Lymphocyte depletion

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Laboratory indicators of tumpr lysis syndrome

Deborah Dalmeida MD

Atleast 2 of the following:

Hyperuricemia

Hyperkalemia

Hyperphosphatemia

Hypocalcemia

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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

Diffuse large B-cell lymphoma (DLBCL)

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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

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

Deborah Dalmeida MD

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List 4 causes for massive splenomegaly

Deborah Dalmeida MD

Primary myelofibrosis

Hairy cell Leukemia

Leishmaniasis

Malaria

Deborah Dalmeida MD

One important clinical feature of tumor lysis syndrome
How do you prevent tumor lysis syndrome?

Deborah Dalmeida MD

Renal failure - due to crystal-induced tissue injury
Aggressive hydration, allopurinol, rasburicase

Deborah Dalmeida MD

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

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

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.

What is the skull xray likely to show?
What is the serum/urine electrophoresis likely to show?
What type of protein is excreted in the urine?

Deborah Dalmeida MD

punched-out defects, 1- 4 cm in diameter
abnormal protein “spikes” in serum or urine electrophoresis- M protein
Free light chains aka Bence Jones proteins

Deborah Dalmeida MD

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

Deborah Dalmeida MD

Immunofixation

Deborah Dalmeida MD

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

Stage IV Deborah Dalmeida MD

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

Solitary Myeloma (Plasmacytoma). Deborah Dalmeida MD

Pruritus and flushing Rhinorrhea Darier sign positive Dermatographism present 1. What enzyme levels would you do to confirm diagnosis? 2. Diagnosis? Deborah Dalmeida MD

1. Serum tryptase- Increased 2. Systemic mastocytosis Deborah Dalmeida MD

WHat do the Howel Jolly bodies contain? Deborah Dalmeida MD

nuclear remnants Deborah Dalmeida MD

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

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

List 4 important events resulting in the finding depicted in the image Deborah Dalmeida MD

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

**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

1. Monoclonal Gammopathy of Uncertain Significance 2. 1% per year Deborah Dalmeida MD

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

1. Pautrier microabscesses 2. Mycosis Fungoides Deborah Dalmeida MD

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

AL (k) type Deborah Dalmeida MD

See the attached image and interpret Deborah Dalmeida MD

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

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

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

**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

Reed-Sternberg cell Deborah Dalmeida MD

Clinicopathologic presentation of Langerhan cell histiocytosis Deborah Dalmeida MD

1. Letterer-Siwe disease 2. eosinophilic granuloma 3. Hand-Schüller-Christian triad 4. Pulmonary Langerhans cell histiocytosis ## Footnote Deborah Dalmeida MD

List the criteria for hypersplenism **Hint:** **H**yper **S**plenism **R**avages **C**ells Deborah Dalmeida MD

Hypercellular or normal marrow Splenomegaly Response to splenectomy Cytopenias Deborah Dalmeida MD

Components of DiGeorge Syndrome Deborah Dalmeida MD

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

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

Peripheral T-Cell Lymphoma Deborah Dalmeida MD

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

TRAP (tartrate-resistant acid phosphatase) stain Deborah Dalmeida MD

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

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

What conditions are thymomas associated with? Deborah Dalmeida MD

Myasthenia gravis Deborah Dalmeida MD

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

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

Hematologic signs of splenectomy Deborah Dalmeida MD

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

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

1. Chronic lymphocytic _leukemia_ OR small lymphocytic _lymphoma_ 2. autoimmune hemolytic anemia (AIHA) Deborah Dalmeida MD

List 4 pathophysiologic factors implicated in multiple myeloma Deborah Dalmeida MD

A. Pathogenic antibodies or antibody fragments B. Bone resorption C. Renal failure D. Suppression of humoral immunity Deborah Dalmeida MD

Identify the stage of Hodgkin's Lymphoma described below: Involvement of a single lymph node region Deborah Dalmeida MD

Stage I Deborah Dalmeida MD

WBC DISORDERS- 2 Flashcards

Lymphomas Disorders of spleen and thymus