Clinical presentation and local status
The disease is characterized by a unicentric growth pattern—the process predictably and strictly sequentially spreads from one group of lymph nodes to another. In 60–80% of cases, the disease debuts with isolated involvement of the cervical lymph nodes.
On palpation, the affected nodes turn into large, dense, and completely painless conglomerates. An important differential sign: they are not fused to each other or to the surrounding tissues. As the disease progresses, deep nodes (intrathoracic, intra-abdominal) become involved in the pathological process, and hepatosplenomegaly develops. In later stages, extranodal dissemination is observed: the tumor infiltrates the lungs, pleura, gastrointestinal tract, bone marrow, and CNS.
Systemic intoxication (B-symptoms)
For accurate staging and selection of therapeutic tactics, the presence or absence of systemic intoxication is critically important. Patients are divided into two categories:
- Category A: the disease proceeds without pronounced signs of intoxication (only lymphadenopathy and nonspecific complaints such as pruritus or fatigue are present).
- Category B: the patient exhibits at least one of the three classical criteria:
- Unexplained fever above 38 °C lasting for more than three days.
- Loss of more than 10% of body weight over the last six months.
- Drenching night sweats.
Ann Arbor staging system
The staging system evaluates three parameters: the number of affected lymphatic areas, their relation to the diaphragm, and the presence of spread beyond the lymphatic system.
- Stage I (localized): only a single lymph node group (or two contiguous groups) is affected, located strictly on one side of the diaphragm.
- Stage II (regional): more than two contiguous or two isolated (non-contiguous) groups are involved, but the process does not cross the diaphragm.
- Stage III (generalized): lymph nodes are affected on both sides of the diaphragm. The disease is still limited to the lymphatic system (including the spleen, thymus, and Waldeyer's ring).
- Stage IV (disseminated): extranodal spread. The tumor affects internal organs (liver, lungs, nervous system, etc.).
Diagnostic workup and treatment principles
The laboratory picture depends on the stage: initially, moderate neutrophilic leukocytosis and eosinophilia are noted, while with progression, leukopenia, anemia, thrombocytopenia, and a sharp increase in ESR develop. Blood biochemistry reveals an increase in fibrinogen and $\alpha_2$-globulins with a drop in albumins.
Morphological verification is the only way to confirm the diagnosis. A lymph node biopsy is performed with histological and immunohistochemical (IHC) examination. The detection of multinucleated giant Berezovsky–Sternberg–Reed cells (4–6 nuclei) against the background of erased nodal architecture is a pathognomonic sign. To assess dissemination, bone marrow trephine biopsy and imaging methods (ultrasound, CT, MRI, PET) are used.
Therapy is carried out in specialized hematology centers. The baseline regimen includes polychemotherapy (cytostatics + glucocorticosteroids) and radiation therapy. In case of relapses, engineered targeted drugs and bone marrow transplantation are used.
The "potatoes in a sack" sign (according to A.A. Kiselev) is a classic metaphor to describe lymph nodes in lymphogranulomatosis: they are large, dense, roll under the skin, and are completely unfused with each other.
Class and exam questions
Can lymphogranulomatosis be diagnosed by ultrasound or blood tests?
No. The only method for definitive verification is histological examination of the substrate (biopsy of the altered lymph node).
What does a unicentric growth pattern mean?
This is a characteristic pathway of spread for Hodgkin lymphoma, in which the process strictly sequentially transitions from one group of lymph nodes to another.
What is the gender and age predisposition in children?
The peak incidence is observed in preschool age, with boys being affected about twice as often as girls.
What else to study on this topic
- Differential diagnosis of systemic and local lymphadenopathy
- Features of polychemotherapy protocols in Hodgkin lymphoma
- Targeted therapy: mechanism of action of brentuximab vedotin
- Indications for bone marrow transplantation in relapses
- Rules for conducting supportive care
Master the entire topic in the Pediatrics course
This is a summary. The course covers everything tested in classes and exams.
- Complete study guide on "Lymphogranulomatosis" and another 3,738 pages of the subject
- Assignments and quizzes after every topic — just like in a colloquium
- AI tutor answers questions on the topic anytime
- 71 video lessons on the subject
Other topics in "Chapter 10. Diseases of the Blood and Blood-Forming Organs"
Prepared by WhiteCoat Pro faculty in accordance with the medical school curriculum. For educational purposes only.