Malignant WBC disorders - MPNs (Quiz 3)

• Fever

• Weight loss

• Increased sweating

What are the major symptoms of leukemia?

Lymphoma

General term for malignancy that starts in the lymphatic system

Benzene

It is used in histopathology and is known for causing leukemia and aplastic leukemia

HTLV-1

Which virus is associated with adult T-cell Leukemia?

HTLV-2

Which virus is associated with Hairy Cell Leukemia?

Short duration and predominantly acute cells

Which of the following defines Acute Leukemia?

WHO

Acute Leukemia is defined as ≥20% blasts in peripheral blood and bone marrow

Cytochemistry

First tool used to distinguish ALL from AML

Immunotyping and Genetic Analysis

Most reliable indicators of cell’s origin to differentiate ALL from AML

Immunophenotyping (Flow Cytometry)

Used to identify cells on the basis of the types of markers or antigens present on the cells surface, nucleus, or cytoplasm

• Helps to identify the lineage of cells

2-5 years of age

The peak of incidence of ALL in children is between:

L1

Most common childhood ALL with best prognosis

• Lymphoblasts are small and homogenous

L2

It is the adult-type ALL

• Lymphoblasts are large and heterogenous

L3

Burkitt-lymphoma type

• Lymphoblasts are large, homogenous and vacuolated

• Rarest subclass

Acute myeloid leukemia (AML)

It is the most common type of leukemia in adults

Normocytic anemia

According to morphology, what subtype of anemia is usually seen in patients suffering with acute leukemia?

M0

AML subtype that is minimally differentiated

• Myeloperoxidase and Sudan black B (NEGATIVE)

M1

Which AML subtype is associated with Chloroma?

Chloroma

Localized tumor masses consisting of myeloblasts

M2

AML subtype with maturation

• Auer rods are present

M3

• Most aggressive type of AML

• Promyelocytes are the predominating cell type

• Faggot cells are present

• Characterized by “butterfly” or “coin-on-coin” nucleus of the promyelocyte

M4

Naegili type of AML

• Pharyngitis may be observed.

• Gingival hyperplasia due to leukemic infiltration may be noted.

• Associated with leukostasis together with M5

M5

Which subtype of AML is Schilling type?

M5a

• Acute monocytic leukemia, poorly differentiated

• >80% monocytic cells

• >80% monoblasts

M5b

• Acute monocytic leukemia, well differentiated

• >80% monocytic cells

• Erythemic myelosis

• Di Guglielmo Syndrome

Acute erythroleukemia is also referred to as:

M6

Which AML subtype represents a proliferation of both immature granulocytic and erythrocytic cells?

• Ring sideroblasts

• Howell-Jolly bodies

What inclusions are found in M6 acute erythroleukemia?

M7

In this AML subtype, dysplastic features are often present in all cell lines.

LAP

• Stains ALP

• Helpful in differentiating chronic myelogenous (CML) from a leukemoid reaction or polycythemia vera.

Peroxidase

Used to differentiate acute myelogenous and monocytic leukemia from acute lymphocytic leukemia

Sudan Black B

• Stains lipids present in the monocytes and granulocytes

• Differentiates AML from ALL

Chloroacetate Esterase

Stains esterases present in the granulocytes

• Used to differentiate granulocytic cells from monocytic cells

Non-Specific Esterase

Stains esterases present in the monocytic cells, macrophages, megakaryocytes and platelets.

• Used to differentiate monocytic leukemias from granulocytic leukemias

Sodium fluoride

Non-specific esterase activity in monocytes can be inhibited by:

Periodic Acid Schiff (PAS)

Stain mucoproteins, glycoproteins, and high molecular weight carbohydrates normally present in almost all blood types except pronormoblast

• Used to help in the diagnosis of Di Guglielmo’s syndrome

• Stains sugars

Magenta red

A positive PAS reaction gives what color?

Acid phosphatase (ACP)

• This stain is helpful in diagnosing hairy cell leukemia (HCL)

• It uses L (+) tartaric acid

Myeloperoxidase

Which stain produces dark brown granules in cytoplasm of granulocytes and monocytes?

Sudan Black B

Which stain produces dark purple black granules in neutrophil precursors?

Naphthol AS-D chloroacetate Esterase

Enzyme activity results in bright red granules in cytoplasm of neutrophils, neutrophil precursors and mast cells

Alpha-naphthyl acetate esterase

Monocytic stain red-brown

Acid phosphatase (ACP)

Activity is indicated by purple to red granules

Toluidine Blue

Useful for recognition of mast cells and tissue basophil

Annexin A

It is specific to hairy cell leukemia and helps to differentiate HCL from related B cell disorders

Burkitt lymphoma

It is an aggressive cancer of mature B cells associated with a fulminant clinical presentation.

Burkitt lymphoma

What type of lymphoma exhibits “starry sky pattern”

Sézary syndrome

It is a disseminated disease with leukemic presentation and skin and lymph node involvement

Hodgkin’s lymphoma

Distinguished from other lymphomas by the presence of “Reed-Sternberg cells”

• Hodgkin cells

• Mummified cells

• Lacunar cells

Neoplastic cells seen in Hodgkin’s disease

Flower cells

Malignant T-cells that are medium to large in size and have accentuated, convoluted nuclei, coarsely clumped chromatin, and deeply basophilic cytoplasm

Chronic lymphocytic leukemia (CLL)

It is the most common type of leukemia in adults in Western countries

Cobblestone or soccer ball

Lymphocyte/lymphoblast chromatin pattern is characterized as:

Basket cell

A common finding on peripheral blood film review in CLL

Diffuse large B cell lymphoma

It is the most common form of Non-Hodgkin’s lymphoma (NHL)

• Aggressive disease that arises either de novo or from transformation of a more indolent form of leukemia or lymphoma

Anaplastic large cell lymphoma (ALCL)

It is a T cell disorder that was associated with large pleomorphic cells

Mantle cell lymphoma (MCL)

It is a lymphoproliferative disorder characterized by medium-sized lymphoid cells with irregular nuclear outlines derived from the follicular mantle zone

Nodal marginal zone lymphoma (NMZL)

Diagnosis of this disease represents a Waste paper basket for disorders that morphologically resemble other low-grade B cell malignancies but lack distinguishing characteristics

Follicular lymphoma

It originates from germinal center B cells and in most cases recapitulates follicular architecture

MPNs

Interrelated clonal hematopoietic stem cell disorders characterized by excessive proliferation of one or more mature myeloid cell lines

Pancytosis

Which abnormality is commonly seen in MPNs?

Chronic myelogenous leukemia (CML)

Stem cell disorder affecting the granulocytic, monocytic, erythrocytic, and megakaryocytic cell lines

• Associated with BCR/ABL1

Dacryocytes

Which cells are seen in Primary myelofibrosis?

• Myelofibrosis

• Essential thrombocythemia

• Polycythemia vera

JAK2 V617F is found in which disorders?

Polycythemia vera

In this disorder, patients exhibit a RUDDY skin coloration due to increase RBC concentration and viscosity of the blood

Gaisbock’s syndrome

Stress polycythemia is also known as:

Hgb >18.5 g/dL in males

Hgb >16.5 g/dL in females

Major diagnostic criteria for PCV

• Hypercellular bone marrow with prominent granulocytic, erythrocytic, and megakaryocytic proliferation

• Decreases serum EPO levels

• Endogenous erythroid colony formation in vitro

Minor diagnostic criteria for PCV