Lymphangioleiomyomatosis

Lymphangioleiomyomatosis

Updated: 11/01/2021

© Jun Wang, MD, PhD

 

General features

  • Cystic destruction with progressive dysfunction of lung
  • Proliferation of lymphatic smooth muscle-like cells
  • Probably originated from perivascular epithelioid cell
  • Most common in reproductive age women
  • Associated with tuberous sclerosis

Key clinical features

  • Most common presentations: Dyspnea, cough, pleural effusion, etc
  • Pneumothorax commonly seen

Key morphological features

Genetic abnormalities

  • TSC1 and TSC2

Diagnosis

  • Radiologic studies: Cystic changes
  • Pathological identification of LAM cells

Markers

  • Positive: HMB45

Treatment

  • Treatments of complications
  • Lung function improvement
  • Lung transplantation

 

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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

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Lymphoid neoplasms

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