Lymphoid and Plasma Cell Neoplasms

Flashcards about Lymphoid and Plasma Cell Neoplasms

In ALL, what cell type does the disease originate from?

Lymphoid blast

What are the normal components of healthy bone marrow?

Red blood cells, white blood cells, platelets, and plasma cells

What abnormalities were observed in the 12-year-old boy with Down syndrome?

Sudden onset of fatigue, pale mucous membranes, and slight splenomegaly

What were the key abnormal laboratory findings in Case 1?

Decreased erythrocytes and hemoglobin, high leukocyte count, blast cells present with Auer rods, and decreased platelets

What cytochemical stains were positive in Case 1?

Sudan black B and naphthol AS-D chloroacetate (NASDCA) esterase

What cytochemical stains were negative in Case 1?

Alpha-naphthyl acetate esterase, PAS, and acid phosphatase

What is the significance of sodium fluoride inhibition in esterase reactions?

Inhibition indicates monocytic origin; no inhibition suggests granulocytic or lymphocytic origin

What were the initial symptoms of the 38-year-old woman in Case 2?

Swollen, bleeding gums for several weeks

What were the physical examination findings in Case 2?

Pale, febrile, with hepatomegaly, splenomegaly, and lymphadenopathy

What were the abnormal lab findings in Case 2?

Decreased erythrocytes and hemoglobin, high leukocyte count, and blast forms in leukocyte distribution.

What cytochemical findings were observed in the bone marrow aspirate of Case 2?

Slightly positive Sudan black B, blue granulation with NASDCA, alpha-naphthyl esterase initially positive but negative with sodium fluoride, and negative PAS reaction

What immunophenotypic markers were positive in Case 2?

CD14, CD64, and CD11c

What cytogenetic abnormality was found in Case 2?

Affect chromosome 11

What condition is indicated by chloroacetate esterase staining neutrophils blue and nonspecific esterase staining monocytes red-brown?

AML-M4

How are leukemias classified?

Acute or chronic based on presenting clinical symptoms, maturity of affected cells, and total leukocyte count

What characterizes Acute Leukemia?

Symptoms of short duration, many immature cells in bone marrow and/or peripheral blood, and total leukocytes varies

What characterizes Chronic Leukemia?

Symptoms of long duration, mostly mature cell forms in bone marrow and/or peripheral blood, and elevated total leukocyte count

What are the most common classes of chronic leukemia?

Lymphoid and plasma cell neoplasms, chronic myelogenous leukemias/myeloproliferative neoplasms, and myelodysplastic/myeloproliferative neoplasms

What is indicative of CLL on a peripheral blood film?

Increased number of mature lymphocytes

What are the general characteristics of Chronic Lymphocytic Leukemia (CLL)?

Most common form of chronic leukemia, mostly a B lymphocyte disease, rare before age 20 and uncommon before age 50, more common in males

What cytogenetic abnormalities are involved in CLL?

t (9;22), del(13), del(q11), del (p17), trisomy 12, trisomy 7, t(8;14)

What is the immunobiology of CLL?

Disease of accumulation of long-lived lymphocytes due to their inability to undergo apoptosis

What is the function of ZAP-70?

An intracellular tyrosine kinase initially discovered because of its role in T and NK-cell signaling but expression leads to poorer prognosis in CLL

What is the Binet staging system for?

Classifying CLL in Europe based on lymphocyte count and areas of swollen lymph nodes, red blood cells, and platelet counts

What is the significance of hypermutations in IgVH genes?

Patients with mutated IgVH domains have better prognosis than those without

What are some clinical signs and symptoms of CLL?

Malaise, low-grade fever, night sweat, weakness, fatigue, anorexia, weight loss, lymphoadenopathy, and hepatosplenomegaly

What are the laboratory findings for CLL?

Leukocytosis, absolute lymphocytosis, peripheral blood film shows 80-90% small lymphocytes with hyper-condensed nuclear chromatin pattern, occasional large lymphoblasts, and smudge cells

What is a key characteristic of the flow cytometry of chronic lymphocytic leukemia?

CD5+CD23+CD20dim+

What are some complications of CLL?

Hyperleukocytosis, anemia and/or thrombocytopenia, hypogammaglobulinemia, immune thrombocytopenic purpura, autoimmune hemolytic anemia, transformation to large cell lymphoma, ALL, or multiple myeloma

When should CLL be treated?

Only patients in Rai III and IV, or Binet C stages or if there are symptoms associated with the disease

What are the general characteristics of Hairy Cell Leukemia (HCL)?

A chronic malignant lymphoproliferative disorder of mature B lymphocytes, more common in males than in females, and more common in patients over 30

What are some morphological and immunophenotypic characteristics of Hairy Cell Leukemia?

Large cell with moderate light blue vacuolated cytoplasm, oval nucleus, strong AP reaction that is not inhibited by tartarate (TRAP ), positive for CD19, CD20, CD22 & CD25

What is the significance of CD103 in diagnosing HCL?

Hairy cell leukemia is positive for this marker in contrast to most other hematologic malignancies which are negative for CD103

What are some signs and symptoms of HCL?

Fatigue, splenomegaly and marrow fibrosis, bleeding, and recurrent infections

What are the treatment options for HCL?

Chemotherapy with cladribine or monoclonals (anti-CD22 recombinant immunotoxin or rituximab [anti-CD20])

What is the significance of TRAP staining?

Used to identify Hairy Cell Leukemia cells

What were the laboratory findings in the 3-year-old girl?

Erythrocyte count and hemoglobin level were below normal, the total leukocyte count was 66.0 × 109/L, blast forms 76%, decreased platelets.

What cytochemical findings are associated with Acute Lymphoblastic Leukemia (ALL)?

Negative myeloperoxidase, strongly positive PAS reaction on the bone marrow lymphoblasts, negative Sudan black B and esterase

What are the typical surface markers for ALL?

TdT, HLA-DR, CD19

What is the cause of Multiple Myeloma?

Cancer of plasma cells (clonal disease)

What is overproduced in Multiple Myeloma?

Monoclonal protein or paraprotein

How is Paraprotein identified on Serum Protein Electrophoresis

A monoclonal Ig (gamma globulin) that is produced in excess by the clonal proliferation of plasma cells.

At what age is Multiple Myeloma common?

Most common in people over 70 yrs

What are the major causes of death from Multiple Myeloma?

Acute infection or renal insufficiency.

What are the CRAB criteria for Multiple Myeloma?

Calcium elevation, Renal insufficiency, Anemia, and Bone disease

What are the symptoms and signs of Multiple Myeloma?

Bone pain and pathologic fractures, renal insufficiency, hypercalcemia, weight loss and night sweating, weakness and fatigue, anemia, pallor, abnormal bleeding, acute infection

What is the key finding in the bone marrow in Multiple Myeloma?

Increased proliferation of plasma cells>10%

What is Rouleaux formation?

Red blood cells are in a stacked formation

How are serum immunoglobulins affected in Multiple Myeloma?

3.5g/dl of IgG or >2.0 g/dl of IgA in serum

How is rouleaux formation detected in the lab?

Increased erythrocyte sedimentation rate

How is Multiple Myeloma staged with the International Staging System?

Based on Beta 2 microglobulin and albumin.

What are the characteristics of monoclonal gammopathy of undetermined significance?

IgG < 3.5 g/dl, IgA < 2.0 g/dl, BJ Protein < 1.0 g/24 H, Bone Marrow Plasma Cells < 10%, No Bone Lesions, No Symptoms

What is Waldenstrom's macroglobulinemia?

one of the malignant monoclonal gammopathies, is a chronic, indolent, lymphoproliferative disorder It is characterized by the presence of a high level of a macroglobulin (immunoglobulin M [IgM]), elevated serum viscosity, and the presence of a lymphoplasmacytic infiltrate in the bone marrow.

What are the clinical features of Waldenstrom's?

Paraproteins (serum immunoglobulin level <3g/dl)., vascular complications

What are the Diagnostic criteria for Waldenström's syndrome?

IgM monoclonal gammopathy of any concentration. • Bone marrow infiltration by pleomorphic B-lineage cells at different stages of maturation, such as small lymphocytes, lymphoplasmacytoid cells, and plasma cells. Total leukocyte count is usually normal, with an absolute lymphocytosis. • Platelet counts are usually normal. Bleeding caused by abnormalities in platelet adhesiveness

What are the main findings to the admission of the 58-year-old librarian?

General malaise and fatigue, with cervical and supraclavicular lymphadenopathy

For the 58-year-old patient admitted with malaise and fatigue, what were her leukocytes results?

Total leukocyte count was 26.5 × 109/L, Bands 6%, Segmented neutrophils 18%, Lymphocytes 75%, Monocytes 1%

What are the key findings in the librarian's bone marrow examination?

Lymphocytic infiltration of approximately 50% of the cells in the marrow

What are lymphomas?

Closely related set of disorders characterized by over- proliferation of one or more types of cells of the lymphoid system such as lymphoreticular stem cells, lymphocytes, reticulum cells, and histiocytes.

How are Lymphomas divided?

Hodgkin Lymphoma (HL) and Non-Hodgkin lymphoma (NHL)

In Hodgkin Lymphoma, what are 2 features they all have?

Men > women and whites > blacks > Asians. Clinically manifest in young adults with no clear risk factors

What cell is characterized as Hodgkin's Disease?

Reed-Sternberg cells: multinucleated giant cells

How does Hodgkin Lymphoma spread?

Spreads through the lymphatic system in an orderly manner originated in lymph nodes of neck (cervical), underarms, groin and abdomen & associates with the presence of Reed-Sternberg cells.

What is the key difference to Hodgkin Lymphoma vs. Non-Hodgkin Lymphoma?

Hodgkin Lymphoma spreads through the lymphatic system vs. NHL may develop anywhere in the body

Hodgkin Lymphomas, what do they demonstrate?

Small numbers of mono- and multinucleated Hodgkin & Reed-Sternberg (HRS) cells [ tumor cells often ringed by normal T- lymphocyte rosettes]Demonstrate an abundant background of mixed inflammatory and accessor cells

In Hodgkin Lymphoma what will PCR demonstrate?

PCR demonstrates clonally rearranged, but non functional Ig genes.

In Hodgkin Lymphoma, what kind of infection pre-dispose the disease?

Epstein-Barr Virus

What are the main differences between a HL vs NHL concerning the HRS cell

Large (15-45µ in diameter) with abundant basophilic/amphophilic cytoplasm and a binucleate or bilobed nucleus

What are the specific surface markers that are positive in HL?

CD30, CD15, CD20, CD79a

What are the chromosome abnormalities frequently seen with HL?

Aneuploidy & hyper-tetra-ploidy (4N)

What are Non-Hodgkin Lymphomas?

A group of clonal lymphoproliferative heterogenous neoplasms which are Most tumors are widely disseminated at diagnosis, requiring systemic therapy

What the common chromosomal abnormalities in NHL?

Frequent chromosomal translocations into Ig gene loci like t(8;14), t(2;8), t(8;22) in Burkitt's Lymphoma

What are the main principles of NHL classification?

Cell size, Nodal architecture, Principle are more aggressive diffuse large cells vs more indolent follicular small cells

New classifications were introduce what are they?

The REAL(revised European American lymphoma) and WHO classifications

What is the Ann Arbor Staging System

Stage I: single lymph node region, Stage II: > 2 lymph node regions on same side of diaphragm, Stage III: both sides of diaphragm involved, and multiple/disseminated foci involved with > 1 extralymphatic organs