Hematopoetic Pathology Flashcards
What is leukocytosis?
Characterized by an elevated white blood cell count.
What is neutrophilia?
- Increased bone marrow production is seen with acute inflammation associated with pyogenic bacterial infection or tissue necrosis
- Increased released from bone marrow storage pool may caused by corticosteroids, stress, or endotoxin
- Increased bands (“left shift”) noted in peripheral circulation
- Reactive changes include Dohle bodies (aggregates of RER), toxic granulations (prominent granules), and cytoplasmic vacuoles of neutrophils
What is eosinophilia?
Increased eosinophils
Occurs with allergies and asthma (Type I hypersensitivity), parasites, drugs (especially in hospitals), and certain skin diseases and cancers (adenocarcinomas, Hodgkin disease)
What is monocytosis?
Increased monocytes
Occurs with certain chronic diseases such as some collagen vascular diseases and inflammatory bowel disease, and with certain infections, especially TB
What is lymphocytosis?
Increased lymphocytes
Occurs with acute (viral) diseases and chronic inflammatory processes
Characteristics of infectious mononucleosis
Acute, self-limited disease, which usually resolves in 4-6 weeks, is an example of a viral disease that causes lymphocytosis. The most common cause is Epstein-Barr virus (a herpes virus) though other viruses can cause it as well (heterophile-negative infectious mononucleosis is most likely due to cytomegalovirus.
What disorders are increased basophils seen?
Chronic myeloproliferative disorders such as polycythemia vera
What is leukopenia?
Characterized by a decreased white blood cell count
What is lymphadenopathy?
Lymph node enlargement due to reactive conditions or neoplasia
Characteristics of acute nonspecific lymphadenitis
- Produces tender enlargement of lymph nodes; focal involvement is seen with bacterial lymphadenitis.
- Microscopically, there may be neutrophils within the lymph node.
- Cat scratch fever (due to Bartonella henselae) causes stellate microabscesses
- Generalized involvement of lymph nodes is seen with viral infections
Characteristics of chronic lymphadenitis
Non-tender enlargement of lymph nodes
Follicular hyperplasia involves B lymphocytes and may be seen with rheumatoid arthritis, toxoplasmosis, and early HIV infections.
Paracortical lymphoid hyperplasia involves T cells and may be seen with viruses, drugs (Dilantin), and SLE.
Sinus histiocytosis involves macrophages and is nonspecific
Characteristics of neoplasia
Nontender enlargement of lymph nodes
The most common tumor is involved lymph nodes is metastatic cancer (e.g., breast, lung, malignant melanoma, stomach and colon carcinoma), which is initially seen under the lymph node capsule
Other important causes of lymphadenopathy are malignant lymphoma and infiltration by leukemias
Chronic lymphocytic leukemia (CLL) vs. Small lymphocytic lymphoma (SLL)
Patients who present with lymph node findings are classified as having SLL.
Patients who present with blood findings are classified as having CLL; 50% of CLL patients also have lymph node involvement
Characteristics of Chronic lymphocytic leukemia (CLL)
CLL is the most indolent of all the leukemias
Mean age at time of diagnosis is age 60
The malginant cells are nonfuncional, so patients develop hypogammaglobulinemia, leading to an increased rick of infections
CLL is associated with warm autoimmune hemolytic anemia (AIHAD) which will cause spherocytes to be observed in peripheral blood
How are CLL and SLL catergorized?
Categorized by the markers present on the cells
Characteristics of B-chronic lymphocytic leukemia
Have B-cell markers, such as CD19 and CD20. One T-cell marker CD5 is present.
Cells are CD23 positive and CD10 negative
SLL occurs only as this type
Characteristics of T-chronic lymphocytic leukemia
Have T-cell markers.
The histology of affected lymph nodes reveals only a diffuse pattern (not nodular) but proliferation centers may also be present
Characteristics of Hairy cell leukemia
B-cell neoplasm that causes indolent disease in middle aged Caucasian men.
There can be a “dry tap” with bone marrow aspiration.
Lymphocytes have “hair-like” cytoplasmic projections; the diagnostic stain is positive tartrate-resistant acid phosphatase (TRAP)
Enlarged spleen due to infiltration of red pulp by malignant cells
Treatment is 2-chloro-seoxyadenosine (2-Cd-A)
Characteristics of follicular lymphoma
Well-differentiated B-cell lymphoma with follicular architecture.
Derived from B lymphocytes
Frequently presents with disseminated disease
Prognosis is better than diffuse lymphoma but doesn’t respond to therapy
What translocation causes follicular lymphoma?
t(14;18)
Involves the heavy chain and BCL2 gene
Characteristics of Diffuse large B-cell lymphoma
High gradelarge B-cell lymphoma with a diffuse growth pattern.
It is an aggressive, rapidly proliferating tumor which may respond to therapy.
Special subtypes include immunodeficiency-associated B-cell lymphomas (EBV) and body cavity large B-cell lymphoma (human herpes virus-8)
Characteristics of small noncleaved lymphoma (Burkitt lymphoma)
High grade B-cell lymphoma
it is composed of interemediate-sized lymphoic cells with a “starry sky” appearance due to numerous reactive tingible-body macrophages
What causes Burkitt’s lymphoma?
t(8;14) translocation
t(2,8) translocation
t(8,22) translocation
What are the two types of burkitt lymphoma?
African types: endemic form
Involvement of mandible or maxilla is characteristic, is associated with Epstein-Barr virus
American type: nonendemic, sporadic form
Involvement of the abdomen (such as bowel, retroperitoneum, or ovaries); has a high incidence in AIDS patients
Characteristics of mantle cell lymphoma (MCL)
Rare B-cell lymphoma in which the tumor cells arise from mantle zone B lymphocytes
What causes mantle cell lymphoma (MCL)?
t(;14) translocation
Involving CCD1 and the heavy chain locus
How to diagnosis mantle cell lymphoma (MCL)?
Positive for:
CD19, CD20, CD5
Negative for:
CD23
Characteristics of marginal zone lymphoma (MALToma)
Diverse group of B-cell neoplasms that arise within lymph nodes,spleen, or extranodal tissue.
It is associated with mucosa-associated lymphoid tissue (MALTomas).
This lesion begins as a reactive polyclonal reaction and may be associated with previous autoimmune disorders or infectious disease (Sjogren disease, Hashimoto thyroiditis, Helicobacter gastritis)
Characteristics of multiple myeloma
Malignant neoplasm of plasma cells
Most common primary tumor arising in the bone marrow of adults
Bence Jones proteins are light chains that are small and can be filtered into the urine
Monoclonal immunoglobulin spike IgG spike
What is cause of multiple myeloma?
t(11;14) translocation cyclin D1;IgH
t(6;14) translocation cyclin D3;IgH
Mnemonic for multiple myeloma
Old CRAB Old- Old age C: hyperCalciumia R: Renal failure A: Anemia B: Bone lytic fractures
Characteristics of plasmacytoma
Solitary myeloma within bone or soft tissue
Within bone: precursor lesion that can later develop into myeloma
Outside bone (extramedullary): usually found within upper respiratory tract
Characteristics of monoclonal gammopathy of undetermined significance (MGUS)
Old name was benign monoclonal gammopathy
M spike
Incidence increases with increasing age
Characteristics of lymphoplasmacytic lymphoma
Also known as Wldenstrom macroglobulinemia
Small lymphocytic lymphoma with plasmacytic differentiation .
M spike present
Russel bodies present (cytoplasmic immunoglobin)
Dutcher bodies present (intranuclear immunoglobin)
Symptoms of lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia)
Hyperviscosity- due to IgM
Visual abnormalities- vascular dilatations and hemorrhages in retina
Neurologic symptoms- headaches and confusion
Bleeding and cryoglobulinemia can be due to abnormal globulins
Characteristics of peripheral T-cell lymphoma, unspecified
“Wastebasket” diagnostic category
Characteristics of adult T-cell leukemia/lymphoma (ATLL)
Malignant T-cell disorder (CD4-T cells) due to HTLV-1 infection
Clinical symptoms: Skin lesions Hypercalcemia Enlarged lymph nodes Heptomegaly Splenomegaly
Microscopic- hyperlobated “4 leaf clover” lymphocytes
Characteristics of mycosis fungoides
Malignant T-cell disorder (CD4+ cells) that has a better prognosis than ATLL
It can present with pruritic erythematous rash
Microscopically, atypical PAS-positive lymphocytes are present in the epidermis; aggreagates of these cells are called Pautrier microabscesses
Characteristics of Sezary syndrome
Like mycosis fungoides
If erythroderma and cerebriform Sezary cells are present in peripheral blood
Characteristics of Hodgkin lymphoma
May present similar to infection (with fever)
Spread is continuous to adjacent node groups
No leukemic state
Extranodal spread is uncommon
Malignant cells are Reed-Sternberg cells (intermixed with reactive inflammatory cells)
Reed-Sternberg cell is a large malignant tumor cell that has a bilobed nucleus with a prominent large inclusion-like nucleolus in each lobe
What is the classification of hodgkin lymphoma?
Lymphocyte-rich Lymphocyte-predominant Mixed cellularity Lymphocyte-depleted Nodular sclerosis
Characteristics of Lymphocyte-rich hodgkin
Rare
Composed primarily of reactive lymphocytes associated with Epstein- Barr virus
Characteristics of lymphocyte-predominant hodgkin
Type: has lymphohistocytic variants (L&H cells, called “popcorn cells” ) and a unique phenotype (CD45+, CD15-, CD30-, CD20+)
Characteristics of mixed cellularity hodgkin
Type: occurs in middle-aged and older males; the increased number of eosinophils is related to IL-5 secretion
Characteristics of lymphocyte-depleted lodgkin
Type: presents with abdominal adenopathy; Reed-Sternberg predominate
Characteristics of nodular sclerosis
type: is the only type with which females>males
Lymph node has broad collagen bands
Reed-Sternberg cell has clear space in the cytoplasm
Characteristics of acute leukemia
Peripheral blood has decreased mature forms and increased immature forms called blasts.
Acute symptoms are due to bone marrow failure: decreased erythrocytes, decrease leukocytes and decreased platelets
Characteristics of acute lymphoblastic leukemia (ALL)
B-cell lineage expresses CD19, CD10 and CD20 markers
T-cell lineage express CD2, CD3, CD5, CD7
Characteristics of acute myelogenous leukemia (AML)
Cancer of the myeloid line of blood cells
Symptoms include fatigue, unusual bleeding and infections
Auer rods (linear condensations of cytoplasmic granules) are characteristic of AML
What causes acute myelogenous leukemia (AML)?
Promyelocytic leukemia has t(15;17) with fusion gene PML/RARA responds to all-transretinoic acid (ATRA); DIC common
AML t(8,21) or inv(16)
Characteristics of myelodysplastic syndromes (MDS)
Classified according to the number of blasts in the marrow.
Dysplastic changes include Pelger-Huet cells (“aviator glasses” nuclei) ring sideroblastss, nuclear budding and “pawn ball” megakaryocytes
MDS are considered preleukemias, so patients are at increased risk of developing acute leukemias
Characteristics of chronic myelogenous leukemia (CML)
Clonal proliferation of pluripotent granulocytic precursor stem cells. In most cases it is associated with a BCR-ABL fusion gene due to a balanced (9;22) translocation (Philadelphia chromosome)
Characteristics of polycythemia vera
Increased erythroid precursors
Increased hemocrit
Increased blood viscosity
Decreased erythropoietin
Increased basophils
Increased eosinophils
High cell turnover
Characteristics of essential thrombocythemia
Increased megakaryocytes in bone marrow.
Peripheral blood smear shows increased platelets, some with abnormal shapes.
Increased leukocytes
Excessive bleeding and occlusion of small vessels
Characteristics of myelofibrosis (MF) with myeloid metaplasia
Marrow fibrosis
Bone marrow aspiration may be a “dry tap”
Enlarged spleen
Peripheral blood smear shows leukoerythroblastosis with teardrop RBCs
Characteristics of Langerhans histiocytosis
Common in children
Affect many sites including skin, bone, CND (diabetes insipidus), and lungs,
Cells are CD1a positive show cytoplasmic Birbeck granules (tennis racket-shaped organelles)
Characteristics of Letterer-Siwe disease
Multisystem variant of langerhans histiocytosis
Marrow involvement can be fatal
Characteristics of Hand-Schuller-Christian
Another variant of langerhans histiocytosis
Triad is calvarial involvement, diabetes insipidus and exophtalmos
Characteristics of unisystem variant of langerhans histiocytosis
Eosinophilic granuloma (most often in bone)
Characteristics of thymomas
Low-grade tumors of the thymic epithelium with many histologic patterns
Characteristics of tue thymic hyperplasia
Enlargement of a histiologically normal thymus; it can occur as a complication of chemotherapy
Characteristics of thymic lymphoid hyperplasia
Shows germinal center hyperplasia
