Mycosis Fungoides and Sezary Syndrome Quiz

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Questions and Answers

What are Russell bodies?

Macrophages found in bone marrow

Inclusions containing DNA in the plasma cells

Globular inclusions of immunoglobulin in the cytoplasm of plasma cells (correct)

Chemokines produced by T helper cells

Which body part is primarily affected by hypercalcemia in Multiple Myeloma?

Liver

Lungs

Bones (correct)

Kidneys

What does the presence of Dutcher bodies indicate?

Significant bone marrow damage

High levels of light chains in urine

Accumulation of immunoglobulin in the cytoplasm

Accumulation of immunoglobulin in the nucleus of plasma cells (correct)

What condition is often associated with hypercalcemia caused by neoplastic plasma cells?

Pathologic fractures Signup and view all the answers

What is Bence Jones proteinuria a result of?

Excretion of free light chains into the urine Signup and view all the answers

What is the primary consequence of increased osteoclast activity in Multiple Myeloma?

Increased bone resorption Signup and view all the answers

Elevated free light chains in plasma typically indicates an excess of which component?

Immunoglobulin light chains Signup and view all the answers

What symptom is commonly associated with severe hypercalcemia?

Confusion and lethargy Signup and view all the answers

Patients with Multiple Myeloma often demonstrate what kind of immunoglobulin abnormality?

Elevated monoclonal Ig levels Signup and view all the answers

What characteristic appearance do plasma cells exhibit when they are neoplastic?

Prominent Golgi apparatus and eccentric nucleus Signup and view all the answers

What is the primary indicator of primary myelofibrosis progression?

Development of extensive marrow fibrosis Signup and view all the answers

What is a definitive characteristic used to diagnose Reed Sternberg cells in Hodgkin Lymphoma?

Presence of multiple nuclei or single multilobular nuclei Signup and view all the answers

Which factor significantly contributes to abnormal blood flow and platelet function in patients with primary myelofibrosis?

Presence of giant platelets Signup and view all the answers

Which feature is often associated with Nodular Sclerosis variant of Hodgkin Lymphoma?

Frequent presence of Lacunar cells Signup and view all the answers

What are the common symptoms associated with sustained congestion in primary myelofibrosis?

Headaches and dizziness Signup and view all the answers

What abnormality indicates a diagnosis of Acute Myeloid Leukemia (AML)?

Presence of more than 20% myeloid blasts in blood Signup and view all the answers

What is the main consequence of extensive marrow fibrosis in myelofibrosis?

Decreased hematopoiesis leading to cytopenia Signup and view all the answers

Which condition is a potential first indication of thrombotic episodes in primary myelofibrosis patients?

Deep vein thrombosis (DVT) Signup and view all the answers

Which mutation is commonly associated with therapy-related Acute Myeloid Leukemia?

t(8,21) Signup and view all the answers

In what form does Myelodysplastic Syndrome (MDS) often present clinically?

Cytopenias and ineffective hematopoiesis Signup and view all the answers

What role does histamine play in the symptoms of primary myelofibrosis?

Leads to intense pruritus and peptic ulceration Signup and view all the answers

What is a common complication associated with organomegaly in primary myelofibrosis patients?

Thrombotic events in portal and mesenteric veins Signup and view all the answers

Which cells are indicative of high risk transformation in Myelodysplastic Syndromes?

Clonal progeny of neoplastic multipotent stem cells Signup and view all the answers

What is commonly found in the marrow of patients with acute promyelocytic leukemia (t(15,17))?

Presence of myeloblasts with Auer rods Signup and view all the answers

What is the relationship between TGF-B and primary myelofibrosis?

Enhances collagen deposition and angiogenesis Signup and view all the answers

Which condition is characterized by an increased proliferation and presence of mutated hematopoietic stem cells?

Myeloproliferative Neoplasm Signup and view all the answers

Which of the following is NOT a characteristic of primary myelofibrosis?

Normal sized platelets Signup and view all the answers

What is a characteristic feature of Lymphocyte-Rich Hodgkin Lymphoma?

High CD15 and CD30 expression Signup and view all the answers

What does the term 'extramedullary hematopoiesis' refer to in primary myelofibrosis?

Hematopoiesis occurring outside the bone marrow Signup and view all the answers

What is the clinical consequence of an increased number of immature myeloid blasts in Acute Myeloid Leukemia?

Ineffective hematopoiesis leading to complications Signup and view all the answers

Study Notes

Mycosis Fungoides/Sezary Syndrome

Tumor involving CD4+ T helper cells primarily affecting the skin
Features cutaneous lesions that progress through: inflammatory premycotic phase, plaque phase, and tumor phase
Neoplastic T cells infiltrate the epidermis and upper dermis, leading to significant abnormalities in the skin
Characteristic of a persistent rash that does not resolve
Sezary syndrome manifests as generalized exfoliative erythroderma
Indolent course with a median survival of approximately 10 years
Histological evidence includes infolding of nuclear membrane and cerebriform appearance of the cells

Plasma Cell Neoplasms

Consist of B cell proliferations generating neoplastic plasma cells
Plasma cells secrete monoclonal immunoglobulin (Ig) or fragments, functioning as tumor markers
Some tumors may exclusively produce light chains or rarely only heavy chains
M component refers to monoclonal Ig present in the blood

Multiple Myeloma

Characterized by IGH locus translocations leading to neoplastic plasma cells
Neoplastic plasma cells produce CCL3, a chemokine that enhances osteoclast formation and bone destruction
Diagnosis requires detection of clonal plasma cells in bone marrow and presence of CRAB symptoms: hypercalcemia, renal dysfunction, anemia, and bone lesions
Predominantly affects men, particularly those aged 65-70, and African Americans
Elevated free light chain levels indicate a skewed balance favoring one light chain
Bence Jones proteins, small free light chains, can be excreted in urine

Clinical Features and Diagnosis

Lytic bone lesions typically occur in the axial skeleton and include punched-out defects leading to pathologic fractures and chronic pain
Hypercalcemia can cause neurologic issues such as confusion and lethargy
The immune system becomes impaired due to decreased production of normal immunoglobulins, leading to recurrent infections
Laboratory findings include elevated Ig levels in blood and/or light chains in urine, alongside the identification of monoclonal Igs in electrophoresis
Common monoclonal Ig identified is IgG, followed by IgA, leading to hyperviscosity syndromes

Smoldering Myeloma vs. MGUS

Smoldering myeloma is characterized by serum M protein >3g/dL or ≥10% clonal plasma cells in the marrow, with a 75% chance of progression to multiple myeloma
MGUS (Monoclonal Gammopathy of Undetermined Significance) typically presents in asymptomatic patients with serum M protein <3g/dL and <10% clonal plasma cells
No evidence of lytic bone lesions or myeloma-related organ complications in MGUS

Symptomatic Plasma Cell Myeloma

Exhibits CRAB features indicating organ and tissue impairment
Neoplastic giant cells, known as Reed-Sternberg cells, are a hallmark of this condition
Differential diagnoses may include infectious mononucleosis and large cell non-Hodgkin lymphoma
Staging is critically important for prognosis, influencing treatment decisions and outcomes.### Hodgkin Lymphoma
Characterized by the presence of Reed-Sternberg cells, which are large cells that may appear as single multilobular nuclei or multiple nuclei resembling owl eyes.
Lacunar cells are a form of Reed-Sternberg cells seen in nodular sclerosis, featuring delicate, folded, multilobate nuclei and pale cytoplasm.
L&H cells indicative of lymphohistiocytic variants have polypoid nuclei, inconspicuous nucleoli, and abundant cytoplasm.
Progression often begins in lymph nodes, later affecting the spleen, liver, bone marrow, and other tissues.
Subtypes include:
- Nodular sclerosis: CD15+, CD30+, EBV-; frequent lacunar cells
- Mixed cellularity: CD15+, CD30+, 70% EBV+; frequent mononuclear and Reed-Sternberg cells
- Lymphocyte-rich: CD15+, CD30+, 40% EBV+; frequent mononuclear Reed-Sternberg cells
- Lymphocyte-depleted: CD15+, CD30+, most EBV+; frequent Reed-Sternberg cells
- Nodular lymphocyte-predominant: CD15-, CD30-, EB-; frequent L&H cells

Myeloid Neoplasms

Acute Myeloid Leukemia (AML)

Caused by acquired oncogenic mutations that hinder myeloid differentiation, leading to an accumulation of immature myeloid forms (blasts).
Replacement of bone marrow with blasts results in marrow failure, causing anemia, thrombocytopenia, and neutropenia.
WHO classification includes subtypes with specific genetic aberrations such as inv(16) and t(15,17).
Auer rods, distinctive granules in myeloblasts, may be noted, particularly in acute promyelocytic leukemia (APL).
Common clinical features include rapid onset of symptoms like anemia, infections, and bleeding due to dysfunctional hematopoiesis.

Myelodysplastic Syndrome (MDS)

Group of clonal stem cell disorders characterized by maturation defects in myeloid progenitors and ineffective hematopoiesis.
Presentation of cytopenias and high risk of transformation to AML.
Associated chromosomal abnormalities include monosomy 5 and 7, as well as deletions of 5q, 7q, and 20q.
Morphological findings reveal dysplastic features in erythroid, granulocytic, and megakaryocytic lineages, such as ring sideroblasts and pseudo-Pelger-Huet cells.

Myeloproliferative Neoplasms

Characterized by increased production of one or more types of blood cells due to mutated, activated tyrosine kinases.
Common features include extramedullary hematopoiesis and potential transformation to acute leukemia or spent phase with marrow fibrosis and cytopenias.

Chronic Myeloid Leukemia (CML)

Driven by the BCR-ABL fusion gene from the Philadelphia chromosome (t(9,22)), leading to increased granulocytic precursors and hypercellular marrow.
Clinical hallmarks include leukocytosis, splenomegaly, and the presence of sea-blue histiocytes.
Progression can lead to an accelerated phase, characterized by anemia and thrombocytopenia, ultimately leading to a blast crisis.

Additional Conditions

Polycythemia Vera

Triggered by activating mutations in JAK2, leading to increased production of red blood cells, granulocytes, and platelets.
Patients exhibit low serum EPO levels, hyperuricemia, and symptoms related to increased red cell mass.
Rare transformation to AML occurs in approximately 1% of cases, with notable features including marked reticulin fiber increase in marrow.### Congestion and Organomegaly
Congestion leads to mild organomegaly, particularly in the spleen and liver due to extramedullary hematopoiesis.
Peripheral blood shows an increased number of basophils and abnormally large platelets.

Progression and Spent Phase

Progression into the spent phase is characterized by extensive marrow fibrosis that displaces hematopoietic cells.
Organomegaly results from extramedullary hematopoiesis, predominantly in the spleen and liver.

Symptoms and Complications

Symptoms include headaches, dizziness, hypertension (HTN), gastrointestinal issues, and thromboses in portal and mesenteric veins.
Hepatic thrombosis can lead to Budd-Chiari syndrome, a complication of blood flow stagnation and deoxygenation.

Role of Basophils

Basophils release histamine, which causes intense pruritus and can lead to peptic ulceration.

Blood Flow and Hemostasis

Abnormal blood flow and impaired platelet function increase risks for bleeding and thrombotic events.
Initial signs of thrombosis can manifest as deep vein thrombosis (DVT), myocardial infarction (MI), or stroke.

Platelet Characteristics

Platelets are notably giant and defective in aggregation, contributing to hemostatic issues.

Primary Myelofibrosis

Activating mutations in JAK2, CALR, or MPL are central to the pathology of primary myelofibrosis.
Transforming growth factor-beta (TGF-B) promotes collagen deposition and angiogenesis in the marrow.

Hallmarks of Primary Myelofibrosis

Development of obliterative marrow fibrosis replaces hematopoietic tissue, leading to cytopenia and reliance on extramedullary hematopoiesis.
Extensive collagen deposition by non-neoplastic fibroblasts impairs normal marrow function and hematopoiesis.

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Description

Test your knowledge on Mycosis Fungoides and Sezary Syndrome, including its classification as a tumor of CD4+ T helper cells and the phases of the disease. This quiz covers cutaneous lesions, the inflammatory premycotic phase, and survival rates. Dive into the details and understand the implications of this complex skin condition.