Lymphoplasmacytic lymphoma

Lymphoplasmacytic lymphoma
Updated: 09/11/2024
© Jun Wang, MD, PhD

General features
  • B cell disorder with features of both low grade lymphoma and myeloma
  • Older patients
  • Frequently involving bone marrow, lymph node, spleen, liver
  • Rarely lytic bone lesion, amyloidosis, renal impairment
  • Autoimmune hemolysis may occur
  • May transform into Diffuse large B cell lymphoma
Clinical presentations
  • Bone marrow abnormality: Anemia, thrombocytopenia, neutropenia
  • Non specific: Lymphoadenopathy, hepatosplenomegaly
  • Hyperviscosity syndrome
  • May be asymptomatic
Key pathogenesis
  • MYD88 mutation resulted in abnormal function of Toll-like receptor and Il-1 receptor signaling pathway
  • Hyperviscosity results in abnormal platelet function
Key Laboratory findings
Key morphological features
  • Small lymphocyte
  • Abnormal plasma cells similar to plasmacytoma
Markers
  • Positive: CD19, CD20, CD138, light chain restriction
Genetic abnormalities
  • MYD88
Negative prognosis indicator
  • Older age
  • Anemia
  • Albumin < 4 g/dl
  • Elevated beta-2-microglobulin


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Contents

Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4.  Benign blood cell disorders Anemia Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas M
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Anemia

Anemia Updated: 07/13/2024 © Jun Wang, MD, PhD   General features Decrease in red cell mass Hemoglobin: Levels of hemoglobin in a specific volume of whole blood Hematocrit: Fraction of whole blood composed of red blood cells Red cell count: Quantity of red cell in a specific volume of whole blood Three major groups based on RBC sizes Microcytic Normocytic Macrocytic Three major causes Blood loss: acute or chronic Increased red cell destruction: hemolysis Reduced production: marrow failure due to genetic abnormality or nutritional deficiency Etiology Genetic abnormality Hemoglobinopathies RBC cytoskeletal abomorlities Enzyme abnormalities Fanconi anemia Nutritional deficiencies: iron, VB12, folate, general malnutrition Physical etiologies: trauma, burns, frostbite, prosthetic valves, etc Chronic diseases: chronic infections, neoplasia, autoimmune disorders, kidney or liver diseases, etc Infectious diseases: hepatitis, sepsis , malaria, etc
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Practice questions Anemia I

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Practice questions Anemia I © Jun Wang, MD, PhD   Abbreviations: Hb: hemoglobin HCT: hematocrit MCV: Mean Corpuscular Volume MCHC: Mean Corpuscular Hemoglobin RDW: Red Cell Distribution Width TIBC: total iron binding capacity   1. Use this case for the next three questions . A 25-year-old woman is brought to the emergency department 2 hours after being involved in a motor vehicle accident. She is conscious but feels dizziness and weakness.   Her blood pressure is 90/60 mmHg, and her heart rate is 120 beats per minute. Physical examination reveals pale skin and mucosa and a 15 cm laceration on her left thigh with significant bleeding. What are most likely the CBC and TIBC results? A. Low Hb, low HCT, high MCV, high RDW, high TIBC B. Low Hb, low HCT, slightly low MCV, normal RDW, normal TIBC C. Low Hb, low HCT, slightly high MCV, normal RDW, normal TIBC D. Normal Hb, normal HCT, normal MCV, normal
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