Bhalani Medicine Pg 132-141
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Questions and Answers

What is the primary genetic feature of the Philadelphia chromosome?

  • An insertion of genetic material
  • A chromosomal duplication
  • A balanced reciprocal translocation (correct)
  • A deletion of a chromosome segment

    • The BCR-ABL1 fusion gene is created by the fusion of the BCR gene on chromosome 9 and the ABL1 gene on chromosome 22.

    False

    In which types of leukemia can the Philadelphia chromosome be found?

    Chronic Myeloid Leukemia, Acute Lymphoblastic Leukemia, Acute Myeloid Leukemia

    The fusion protein BCR-ABL1 leads to excessive cell __________ and reduced apoptosis.

    <p>proliferation</p> Signup and view all the answers

    Match the leukemia types with their association with the Philadelphia chromosome:

    <p>Chronic Myeloid Leukemia (CML) = Commonly associated Acute Lymphoblastic Leukemia (ALL) = Approximately 25-30% in adults Acute Myeloid Leukemia (AML) = Occasionally associated Acute Lymphoblastic Leukemia (ALL) in Pediatrics = 2-10% cases</p> Signup and view all the answers

    What is the primary mode of action of low-molecular weight heparins (LMWH)?

    <p>Inhibiting activated factor X (Xa)</p> Signup and view all the answers

    Low-molecular weight heparins are more immunogenic than conventional heparin.

    <p>False</p> Signup and view all the answers

    Name one advantage of administering low-molecular weight heparins (LMWH).

    <p>Can be administered subcutaneously once or twice per day.</p> Signup and view all the answers

    The molecular weights of low-molecular weight heparins (LMWH) range from _____ to _____ Daltons.

    <p>3000, 8000</p> Signup and view all the answers

    Match the following low-molecular weight heparins (LMWH) to their common names:

    <p>Enoxaparin = Lovenox Dalteparin = Fragmin Tinzaparin = Innohep</p> Signup and view all the answers

    At which age range does Hodgkin lymphoma show a bimodal peak incidence?

    <p>15-35 years and 45-70 years</p> Signup and view all the answers

    Alcohol induced discomfort in the lymph nodal region is common in Non-Hodgkin lymphoma.

    <p>False</p> Signup and view all the answers

    What type of involvement is more common in Non-Hodgkin lymphoma regarding ectopic lymph nodes?

    <p>Extranodal involvement</p> Signup and view all the answers

    In Hodgkin lymphoma, neoplastic cells known as __________ form a minor tumor cell mass.

    <p>Reed-Sternberg cells</p> Signup and view all the answers

    Match the characteristics with Hodgkin and Non-Hodgkin lymphoma:

    <p>Well localized at diagnosis = Hodgkin lymphoma Commonly involves mesenteric nodes = Non-Hodgkin lymphoma Orderly spread = Hodgkin lymphoma Majority of cells are neoplastic = Non-Hodgkin lymphoma</p> Signup and view all the answers

    Which of the following is classified as a precursor lymphoid neoplasm?

    <p>B lymphoblastic leukemia/lymphoma</p> Signup and view all the answers

    The recommended first-line therapy for low-grade non-Hodgkin's lymphoma is three monoclonal antibodies alone.

    <p>False</p> Signup and view all the answers

    What chemotherapy regimen is typically used for high-grade non-Hodgkin's lymphoma?

    <p>CHOP</p> Signup and view all the answers

    The combination of chlorambucil is typically used for _____ therapy in low-grade NHL.

    <p>oral</p> Signup and view all the answers

    Which monoclonal antibody is commonly used in combination with chemotherapy for the treatment of high-grade NHL?

    <p>Rituximab</p> Signup and view all the answers

    Match the lymphoma type with its management approach:

    <p>Low-grade NHL = Rituximab in combination with chemotherapy High-grade NHL = CHOP regimen Localized stage I disease = Radiotherapy Relapsed chemosensitive disease = Autologous bone marrow transplantation</p> Signup and view all the answers

    The CHOP-R regimen includes cyclophosphamide, doxorubicin, vincristine, and _____ as key components.

    <p>prednisone</p> Signup and view all the answers

    What is the role of Rituximab in the treatment of diffuse large-cell lymphoma with stage II or greater?

    <p>Improved overall survival when used with CHOP chemotherapy.</p> Signup and view all the answers

    What is a common neurological manifestation associated with multiple myeloma?

    <p>Sensory and/or motor loss</p> Signup and view all the answers

    Multiple myeloma primarily affects females more than males.

    <p>False</p> Signup and view all the answers

    In which decades is the peak incidence of multiple myeloma typically observed?

    <p>6th to 7th decades</p> Signup and view all the answers

    Patients with osteosclerotic myeloma may exhibit features of ________ syndrome.

    <p>POEMS</p> Signup and view all the answers

    Match the following clinical findings with their associated issues in multiple myeloma:

    <p>Bone pain = Marrow infiltration Renal failure = Monoclonal protein Increased infections = Immune deficiency Anemia = Marrow infiltration</p> Signup and view all the answers

    What is a characteristic feature of the immune system affected by multiple myeloma?

    <p>Thrombocytopenia</p> Signup and view all the answers

    Hyperviscosity syndrome rarely affects the central nervous system (CNS).

    <p>True</p> Signup and view all the answers

    List two symptoms associated with hyperviscosity syndrome in multiple myeloma.

    <p>Confusion, headache</p> Signup and view all the answers

    Which of the following is a major disorder associated with disseminated intravascular coagulation (DIC)?

    <p>Meningococcemia</p> Signup and view all the answers

    DIC can only occur as an acute condition and cannot be chronic.

    <p>False</p> Signup and view all the answers

    What laboratory finding indicates a decreased platelet count in DIC?

    <p>Utilization in microthrombi</p> Signup and view all the answers

    In cases of DIC, the _______ time is often prolonged due to consumption of clotting factors.

    <p>Prothrombin</p> Signup and view all the answers

    Match the following abnormal laboratory values with their corresponding conditions in DIC:

    <p>Platelet count = Decreased Fibrin degradation products = Elevated Prothrombin time = Prolonged APTT = Prolonged</p> Signup and view all the answers

    What is a primary management approach for DIC?

    <p>Control the underlying cause</p> Signup and view all the answers

    Elevated D-dimer levels are specific for diagnosing DIC.

    <p>True</p> Signup and view all the answers

    Name one clinical feature commonly observed in acute DIC.

    <p>Bleeding from mucous membranes</p> Signup and view all the answers

    Patients with DIC may exhibit symptoms related to _______ or hemorrhagic diathesis.

    <p>tissue hypoxia</p> Signup and view all the answers

    What should be monitored when administering low doses of heparin for DIC management?

    <p>Bleeding correction</p> Signup and view all the answers

    What is a key genetic factor that causes paroxysmal nocturnal hemoglobinuria (PNH)?

    <p>Mutation in the pig-A gene</p> Signup and view all the answers

    Thrombocytopenia is a common finding in paroxysmal nocturnal hemoglobinuria.

    <p>True</p> Signup and view all the answers

    What symptom is commonly associated with dark urine in PNH patients?

    <p>Hemoglobinuria</p> Signup and view all the answers

    In PNH, red blood cells lack inhibitors such as decay-accelerating factor (CD55) and ________.

    <p>CD59</p> Signup and view all the answers

    Match the clinical features with their descriptions:

    <p>Intravascular Hemolysis = Dark-colored urine due to hemoglobinuria Thrombosis = Commonly affects hepatic and cerebral veins Iron loss = Leads to iron deficiency anemia Bone marrow changes = Can be hypoplastic or aplastic</p> Signup and view all the answers

    Which test is used to assess red blood cell fragility in acidic conditions?

    <p>Ham's Acidified Serum Test</p> Signup and view all the answers

    PNH primarily affects children and women more than men.

    <p>False</p> Signup and view all the answers

    What is an effective treatment option for PNH?

    <p>Eculizumab</p> Signup and view all the answers

    A reduced NAP score indicates a deficiency in ________ linked proteins.

    <p>GPI</p> Signup and view all the answers

    What complications can develop in PNH patients apart from symptomatic manifestations?

    <p>Acute myelogenous leukemia</p> Signup and view all the answers

    What percentage of non-Hodgkin lymphoma cases are of B-cell origin?

    <p>80%</p> Signup and view all the answers

    The peak incidence of non-Hodgkin lymphoma occurs around the age of 40.

    <p>False</p> Signup and view all the answers

    Name one infectious agent that is associated with non-Hodgkin lymphoma.

    <p>Epstein-Barr virus</p> Signup and view all the answers

    Non-Hodgkin lymphoma most commonly presents with __________ lymph node enlargement.

    <p>painless firm</p> Signup and view all the answers

    Match the types of non-Hodgkin lymphoma with their associated grades:

    <p>Follicular lymphoma = Low-grade with good prognosis Diffuse lymphoma = High-grade with poor prognosis Bone marrow involvement = More common in low-grade NHL T-cell lymphoma = More common in extranodal involvement</p> Signup and view all the answers

    Which condition is NOT considered an acquired immune disorder associated with non-Hodgkin lymphoma?

    <p>Wiskott-Aldrich syndrome</p> Signup and view all the answers

    High uric acid levels are only a concern in low-grade non-Hodgkin lymphomas.

    <p>False</p> Signup and view all the answers

    What does immunophenotyping help to distinguish in lymphomas?

    <p>T-cell and B-cell tumors</p> Signup and view all the answers

    The staging system used for non-Hodgkin lymphoma is known as __________ classification.

    <p>Ann Arbor</p> Signup and view all the answers

    Which of the following is a common presentation of non-Hodgkin lymphoma?

    <p>Painless firm lymph node enlargement</p> Signup and view all the answers

    Which of the following is an immediate immunological complication of blood transfusion?

    <p>Acute hemolytic transfusion reactions</p> Signup and view all the answers

    Febrile non-hemolytic reactions typically occur at the beginning of blood transfusions.

    <p>False</p> Signup and view all the answers

    What is a common symptom of anaphylactic reactions during a blood transfusion?

    <p>Difficulty breathing</p> Signup and view all the answers

    Transfusion-associated graft versus host disease (TA-GVHD) typically occurs ____ to ____ days post-transfusion.

    <p>8, 10</p> Signup and view all the answers

    Match the delayed reactions to their descriptions:

    <p>Alloimmunization = Development of antibodies against transfused antigens Delayed hemolytic transfusion reactions (DHTR) = Occurs 3-21 days post transfusion Transfusion-associated graft versus host disease (TA-GVHD) = Immune response from donor T lymphocytes Post-transfusion purpura = Immune-mediated thrombocytopenia in parous women</p> Signup and view all the answers

    What is a potential consequence of circulatory overload during a blood transfusion?

    <p>Fluid overload in the lungs</p> Signup and view all the answers

    Iron overload due to frequent blood transfusions is known as transfusion hemosiderosis.

    <p>True</p> Signup and view all the answers

    Name one infection that can potentially arise from blood transfusions.

    <p>Hepatitis</p> Signup and view all the answers

    Allergic reactions during blood transfusions may manifest as an itchy __________.

    <p>rash</p> Signup and view all the answers

    Which complication is associated with antibodies against human platelet antigens?

    <p>Post-transfusion purpura</p> Signup and view all the answers

    Which of the following symptoms is commonly associated with anemia in acute leukemia?

    <p>Shortness of breath on effort</p> Signup and view all the answers

    Neutropenia in acute leukemia can lead to life-threatening infections.

    <p>True</p> Signup and view all the answers

    What is the primary goal of curative intent therapy in acute leukemia?

    <p>To cure the patient</p> Signup and view all the answers

    In acute leukemia, thrombocytopenia can present as ______ manifestations.

    <p>bleeding</p> Signup and view all the answers

    Match the following conditions with their associated symptoms:

    <p>Anemia = Shortness of breath Neutropenia = Life-threatening infections Thrombocytopenia = Bleeding manifestations Leukostasis = Headache and confusion</p> Signup and view all the answers

    What is the most common cause of bone pain in patients with acute leukemia?

    <p>Marrow expansion</p> Signup and view all the answers

    Leukemic meningitis is a common occurrence in acute leukemia.

    <p>False</p> Signup and view all the answers

    What do Auer rods indicate in a peripheral blood smear?

    <p>Presence of myeloblasts</p> Signup and view all the answers

    Acute myeloid leukemia may lead to ______ due to disseminated intravascular coagulation.

    <p>hemorrhagic diathesis</p> Signup and view all the answers

    Which factor increases the risk of infections in patients with acute leukemia?

    <p>Neutropenia</p> Signup and view all the answers

    Supportive care is unnecessary when administering curative treatment for acute leukemia.

    <p>False</p> Signup and view all the answers

    What are chloromas in acute leukemia?

    <p>Localized solid tumor masses</p> Signup and view all the answers

    The presence of ______ leukemic blast cells in CSF characterizes leukemic meningitis.

    <p>myeloblast</p> Signup and view all the answers

    Which type of acute leukemia is associated with the best survival rates?

    <p>Childhood acute lymphoblastic leukemia</p> Signup and view all the answers

    Elevated serum LDH levels are found in patients with acute leukemia.

    <p>True</p> Signup and view all the answers

    Study Notes

    WHO Classification of Lymphoid Neoplasms (2016)

    • Lymphoid neoplasms are categorized based on cell origin (precursor or mature) and lineage (B-cell, T-cell, or NK-cell).
    • Precursor lymphoid neoplasms include B lymphoblastic leukemia/lymphoma and T lymphoblastic leukemia/lymphoma.
    • Mature B-cell neoplasms include chronic lymphocytic leukemia/small lymphocytic lymphoma, monoclonal B-cell lymphocytosis, B-cell prolymphocytic leukemia, splenic B-cell marginal zone lymphoma, hairy cell leukemia, lymphoplasmacytic lymphoma, heavy chain disease, plasma cell neoplasm, follicular lymphoma, mantle cell lymphoma, diffuse large B-cell lymphoma, and Burkitt lymphoma.
    • Mature T and NK cell neoplasms include T-cell prolymphocytic leukemia, T-cell large granular lymphocytic leukemia, mycosis fungoides, Sezary syndrome, peripheral T-cell lymphoma (NOS), angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma, adult T-cell leukemia/lymphoma, and extranodal NK/T-cell lymphoma (nasal type).

    Management of Non-Hodgkin's Lymphoma (NHL)

    • Low-grade NHL can be managed with radiotherapy, chemotherapy, monoclonal antibody therapy, and bone marrow transplantation.

    • Radiotherapy is used for localized stage I disease.

    • Chemotherapy includes oral chlorambucil (not curative) and intensive intravenous chemotherapy for younger patients. Bendamustine rituximab is recommended as first-line therapy.

    • Three monoclonal antibodies (rituximab, 131I-tositumomab, and 90Y-ibritumomab) are approved for NHL treatment. Rituximab (R) alone or with chemotherapy (e.g., R-CVP) is recommended as first-line therapy.

    • Bone marrow transplantation is an option for low-grade NHL treatment.

    • High-grade NHL is primarily treated with intravenous combination chemotherapy, most commonly using the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone).

    • Radiotherapy is indicated for residual localized disease after chemotherapy and spinal cord compression syndromes.

    • Humanized monoclonal antibody therapy, such as rituximab (R), improves overall survival when combined with CHOP chemotherapy. R-CHOP is recommended as first-line therapy for stage II or greater diffuse large-cell lymphoma.

    • Autologous bone marrow transplantation is used for patients with relapsed chemosensitive disease.

    Philadelphia Chromosome (Ph)

    • The Philadelphia chromosome is an acquired chromosomal abnormality found in hematopoietic stem cells.
    • It is characterized by a balanced reciprocal translocation between chromosomes 9 and 22 (t(9;22)).
    • This translocation results in the fusion of the breakpoint cluster region (BCR) gene on chromosome 22q with the ABL1 gene on chromosome 9q, creating the BCR-ABL1 fusion oncogene.
    • The BCR-ABL1 oncoprotein has uncontrolled tyrosine kinase activity, leading to excessive cell proliferation and reduced apoptosis.
    • The Philadelphia chromosome is not exclusive to Chronic Myeloid Leukemia (CML), It can also be found in Acute Lymphoblastic Leukemia (ALL) and, occasionally, Acute Myeloid Leukemia (AML).
    • Patients with the Philadelphia chromosome (Ph+) and BCR-ABL1 fusion gene have better survival rates and therapeutic responses.

    Multiple Myeloma

    • Multiple myeloma is a cancer of plasma cells, characterized by an uncontrolled proliferation of these cells, leading to bone marrow infiltration.
    • The disease primarily affects adults, particularly those in the 6th and 7th decades of life.
    • Symptoms include bone pain (especially in the vertebrae, skull, sternum, ribs, and clavicle), fatigue, anemia, infections, and renal impairment.
    • Osteosclerotic myeloma, or POEMS syndrome, is a rare variant of multiple myeloma characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.

    Clinical Features of Multiple Myeloma

    • Multiple myeloma can affect multiple organ systems, resulting in various clinical features.
    • Bone marrow infiltration leads to bone pain, fracture, and osteoporosis.
    • Monoclonal protein production can cause renal failure, hyperviscosity, and amyloidosis.
    • Anemia is a common symptom, and immune deficiency leads to increased susceptibility to infections.
    • Neurological manifestations can include sensory and motor loss due to spinal cord compression, and can present with backache, weakness, sensory loss, and urinary retention.
    • Other complications include hypercalcemia, bleeding tendency, and cryoglobulinemia.

    Low-Molecular Weight Heparins (LMWH)

    • LMWHs are biologically active forms of conventional heparin with molecular weights ranging from 3000 to 8000 Daltons.
    • They act as anticoagulants primarily by inhibiting activated factor X (Xa), rather than activated factor II (IIa).
    • Advantages of LMWHs include subcutaneous administration once or twice per day, predictable pharmacokinetics, no need for aPTT monitoring, reduced immunogenicity, lower risk of thrombocytopenia, and suitability for outpatient treatment of deep vein thrombosis (DVT).
    • A disadvantage is higher cost compared to unfractionated heparin.
    • Commonly available LMWHs include enoxaparin, dalteparin, and tinzaparin.

    Hodgkin Lymphoma vs. Non-Hodgkin Lymphoma

    • Hodgkin lymphoma and non-Hodgkin lymphoma are distinct diseases with important differences summarized in Table 8.58.

    Paroxysmal Nocturnal Hemoglobinuria (PNH)

    • PNH is a rare acquired non-malignant, genetic disorder caused by a mutation in the hematopoietic stem cell.
    • The mutation results in red blood cells lacking the GPI-linked proteins CD55 and CD59, making them sensitive to complement-mediated intravascular hemolysis.
    • The severity of hemolysis correlates with the number of abnormal red blood cells.
    • Commonly affects adults, usually middle-aged individuals, and both sexes are equally affected.

    Clinical Features of Paroxysmal Nocturnal Hemoglobinuria (PNH)

    • Classic symptoms include dark-colored urine (especially in the morning) due to hemoglobinuria, abdominal pain, dysphagia, erectile dysfunction, pulmonary hypertension, and renal insufficiency.
    • Mild jaundice and hepatosplenomegaly are often present.
    • Thrombosis, particularly involving hepatic, portal, or cerebral veins, is a significant complication and a common cause of death.
    • Patients may progress to aplastic anemia.

    Disseminated Intravascular Coagulation (DIC)

    • DIC is a widespread acute or chronic thrombohemorrhagic disorder occurring as a secondary complication of various disorders.
    • It is a complex process involving widespread activation of coagulation and fibrinolysis, leading to microthrombi formation and depletion of clotting factors, resulting in a bleeding diathesis.

    Causes of Disseminated Intravascular Coagulation (DIC)

    • Infections: Gram-negative bacterial sepsis, meningococcemia, fungi, viruses, Rocky Mountain spotted fever, malaria.
    • Obstetric complications: Retained dead fetus, septic abortion, abruptio placentae, amniotic fluid embolism, toxemia, and preeclampsia.
    • Neoplasms: Carcinomas of the pancreas, prostate, lung, stomach, acute promyelocytic leukemia.
    • Massive tissue injury: Trauma, burns, fat embolism, surgery.
    • Vascular disorders: Aortic aneurysm, giant hemangioma.
    • Miscellaneous: Snakebite, liver disease, acute intravascular hemolysis, shock, heat stroke, vasculitis.

    Laboratory Findings in Disseminated Intravascular Coagulation (DIC)

    • Screening assays: Decreased platelet count, prolonged prothrombin time, prolonged APTT, increased thrombin time, decreased plasma fibrinogen, presence of schistocytes (fragmented red blood cells) in peripheral smear.
    • Confirmatory tests: Elevated FDP (fibrin degradation products) levels, elevated D-dimer levels.

    Management of Disseminated Intravascular Coagulation (DIC)

    • Control or eliminate the underlying cause.
    • Correct precipitating factors (acidosis, dehydration, sepsis, hypoxia).
    • Manage hemorrhagic symptoms with blood transfusions (platelet concentrates, FFP, cryoprecipitate, red blood cells).
    • Heparin (low doses for continuous infusion) can be used for thrombotic manifestations.
    • Antifibrinolytic drugs, such as EACA and tranexamic acid, can prevent fibrin degradation but increase the risk of thrombosis when used with heparin.

    Clinical Features of Disseminated Intravascular Coagulation (DIC)

    • Symptoms depend on the underlying disorder's nature, intensity, and duration.
    • Bleeding (ecchymoses, petechiae, mucosal bleeding, bleeding at venipuncture sites) is the most common clinical feature in acute DIC.
    • Microvascular thrombi can cause ischemic necrosis of organs, leading to gangrene or hemorrhagic necrosis of the skin, particularly in chronic underlying diseases.
    • Waterhouse-Friderichsen syndrome (adrenal hemorrhage) and Trousseau sign (migratory venous thrombosis in cancers) are associated with DIC.
    • Multiorgan dysfunction syndrome (MODS) can occur due to bleeding into various organs.

    Acute Myeloid Leukemia (AML)

    • AML is a type of leukemia characterized by the uncontrolled growth of abnormal myeloblasts in the bone marrow.
    • Clinical features are often similar to Acute Lymphoblastic Leukemia (ALL).

    Clinical Features of Acute Myeloid Leukemia (AML)

    • Nonspecific flu-like symptoms are the most common initial presentations.
    • Bone marrow failure leads to anemia, neutropenia, and thrombocytopenia, resulting in fatigue, weakness, infections, and bleeding manifestations.
    • Bone pain and sternal tenderness can occur due to marrow expansion and infiltration of the subperiosteum.
    • Leukostasis (stasis of blood flow) can cause headache, confusion, visual disturbances, dyspnea, chest pain, and acute respiratory distress syndrome.
    • Coagulopathy (DIC and primary fibrinolysis) may lead to hemorrhagic diathesis.
    • Extramedullary leukemic infiltration can involve the gums, skin, liver, spleen, lymph nodes, and meninges.

    Investigations for Acute Myeloid Leukemia (AML)

    • Blood count: Low hemoglobin, elevated total leukocyte count (usually less than 100 × 109/L), often with leukopenia, and markedly decreased platelet count.
    • Peripheral blood smear: Shows numerous blast cells and Auer rods (rod-shaped red inclusions in the cytoplasm of myeloblasts).

    Management of Acute Myeloid Leukemia (AML)

    • Palliative therapy: Provides symptomatic relief and includes medications, transfusions, and supportive care.
    • Curative therapy: Aimed at achieving complete remission and long-term survival. This usually involves intensive chemotherapy and, in some cases, bone marrow transplantation.

    Leukemia Treatment

    • Curative intent aims to eliminate the leukemia, often involving chemotherapy and possibly a stem cell transplant.
    • Active therapy may involve specific treatments targeting the leukemia or supportive care to manage symptoms.
    • Supportive therapy is crucial for both curative and palliative approaches.
    • Treatment of anemia includes frequent red blood cell transfusions to maintain hemoglobin levels above 10 g/dL.
    • Treatment of thrombocytopenia involves platelet transfusions to prevent or control bleeding.
    • Infection prevention and treatment involves handwashing, isolation, and appropriate antibiotics for confirmed infections.
    • Continuous monitoring of liver, kidney, and clotting functions is essential for managing potential complications.
    • Treatment of hyperleukocytosis can involve chemotherapy, hydroxyurea, or leukapheresis to reduce high white blood cell counts.
    • Bone marrow transplantation is considered for specific leukemia types, including high-risk ALL and AML in first remission, particularly for patients under 60 years old.
    • Survival rates vary widely depending on the specific leukemia type, ranging from over 90% to less than 10%.

    Non-Hodgkin Lymphoma (NHL)

    • Etiology: NHL development is associated with a variety of factors.
      • Genetic Factors: Family history of lymphoma and inherited syndromes like ataxia-telangiectasia and Wiskott-Aldrich syndrome.
      • Acquired Immune Disorders: Immunosuppression from organ transplantation, AIDS, rheumatoid arthritis, systemic lupus erythematosus, Sjögren syndrome, and Hashimoto thyroiditis.
      • Infectious Agents: Epstein-Barr virus (EBV) associated with Burkitt and Hodgkin lymphoma, Human T-lymphotropic virus type 1 (HTLV-1) associated with adult T-cell leukemia/lymphoma, Human herpesvirus 8 (HHV-8) associated with primary effusion lymphoma, Hepatitis C virus (HCV) associated with lymphoplasmacytic lymphoma and splenic marginal zone lymphoma, and Helicobacter pylori associated with gastric lymphomas.
      • Occupational and Environmental Exposure: Ionizing radiation, herbicides, organic solvents, hair dyes, UV light, high-fat diets, nitrates in drinking water, and heavy smoking.

    NHL Pathology and Classification

    • Grading: Prognosis is influenced by the size of lymphoid cells. Small cells indicate low-grade disease, while large cells signify high-grade disease.
      • Follicular lymphomas: Usually low-grade with favorable prognosis.
      • Diffuse lymphomas: Primarily high-grade, associated with poor prognosis.
    • WHO Classification: Requires immunophenotyping, cytogenetics, FISH, and antigen receptor gene rearrangement studies.

    NHL Clinical Features

    • Age: NHL can occur at any age, but the peak incidence falls around 60 years.
    • Presentation: Painless firm lymph node enlargement, or symptoms related to lymph node mass.
    • Extranodal Involvement: More common in T-cell lymphomas. May involve bone marrow, gut, thyroid, lung, skin, testis, brain, and rarely, bone.
    • Bone Marrow Involvement: More frequent in low-grade NHL, potentially leading to cytopenia.
    • Primary Extranodal Lymphomas: Present with soft tissue masses and symptoms based on the affected site, including Waldeyer's ring and epitrochlear lymph nodes.
    • Pressure Effects: May cause obstruction of organs (gut, ascites, SVC), spinal cord compression.
    • Hepatosplenomegaly: Enlargement of liver and spleen.
    • Mediastinal Mass: Common in lymphoblastic lymphoma

    NHL Clinical Staging

    • Ann Arbor Classification: Used for both Hodgkin and non-Hodgkin lymphoma.
    • "B" symptoms (weight loss, fever, sweats): Do not reliably predict prognosis in NHL.

    NHL Investigations

    • Peripheral Blood: May show moderate anemia with significant bone marrow involvement. Typically, blood counts are normal, but lymphocytosis and splenomegaly can occur.
    • Bone Marrow Aspiration and Trephine Biopsy: Performed early due to the frequent occurrence of marrow involvement in NHL.
    • Immunophenotyping: Uses flow cytometry and immunohistochemistry to differentiate T- and B-cell tumors (e.g., CD45, CD20, CD3).
    • Immunoglobulin Determination: Some lymphomas are associated with IgG or IgM paraproteins.
    • Uric Acid Levels: Important in aggressive high-grade NHLs as high levels can lead to renal failure.
    • HIV Testing: Necessary if suspected.
    • Serum Levels of LDH, β2-macroglobulin, and Other Serum Proteins: May be measured depending on the specific situation.

    Transfusion Reactions

    • Transfusion Complications: Categorized as immunological or non-immunological:
      • Immunological Reactions:
        • Immediate:
          • Acute hemolytic transfusion reaction: Recipient's immune system attacks transfused red blood cells due to incompatibility.
          • Febrile non-hemolytic reaction: Common reaction, often caused by leukocytes or antibodies in donor blood.
          • Allergic reactions (Urticaria): Less severe reaction, characterized by an itchy rash.
          • Anaphylactic reactions: Severe allergic reaction triggered by IgA in donor blood, leading to breathing difficulties, hypotension, and shock.
          • Transfusion-related acute lung injury (TRALI): Occurs within 6 hours, characterized by fever, difficulty breathing, and lung infiltrates on chest X-ray.
        • Delayed:
          • Alloimmunization: Development of antibodies against antigens in transfused blood, potentially causing problems with subsequent transfusions.
          • Delayed hemolytic transfusion reactions (DHTR): Occur 3-21 days after transfusion, due to sensitization to antigens in prior transfusions.
          • Transfusion-associated graft-versus-host disease (TA-GVHD): Rare but fatal complication, occurring 8-10 days after transfusion, characterized by fever, rash, liver dysfunction, diarrhea, and pancytopenia.
          • Post-transfusion purpura: Immune-mediated thrombocytopenia, causing bleeding and ineffective platelet transfusions.
      • Non-Immunological Reactions:
        • Immediate:
          • Circulatory overload: Occurs with rapid infusion of large blood volumes.
          • Air embolism: Air entering the bloodstream.
        • Late:
          • Iron overload (Transfusion hemosiderosis): Iron accumulation from frequent transfusions.
          • Thrombophlebitis: Inflammation of a vein.
          • Infections: Hepatitis, HIV, malaria, cytomegalovirus.
    • Treatment: Specific treatment depends on the complication. May involve stopping the transfusion, administering medications, or plasmapheresis.
    • Prevention: Some complications, like TA-GVHD, can be prevented by using irradiated blood products.

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