Diagnosis based on
Symptoms and signs: Systemic symptoms include fever, night sweats, and weight loss. Local manifestations include painless superficial lymph node enlargement. Invasion of the pharyngeal lymphatic ring presents with foreign body sensation and pain during swallowing. Invasion of the mediastinum results in superior vena cava compression syndrome, manifested by swelling of the face, face and both upper limbs, and shortness of breath. Invasion of the abdominal organs results in hepatosplenomegaly, jaundice, and even liver failure. Involvement of retroperitoneal lymph nodes presents with epigastric fullness and lumbar soreness. Invasion of the digestive tract shows loss of appetite, abdominal pain, diarrhea, abdominal masses, intestinal obstruction and bleeding.
Invasion of the lung parenchyma and pleura occurs with coughing, hemoptysis, chest tightness, and breath-holding. Invasion of the skin shows subcutaneous nodules, infiltrative plaques, ulcers, rashes, and papules. Invasion of the nervous system causes hemiplegia, urinary retention, lower limb convulsions, numbness and soreness in the sacrococcygeal region and difficulty walking.
Routine tests: Complete blood count, biochemistry, head/neck/thorax/abdomen/pelvis CT, cardiac function, PET scan or 67 scan, LDH, β2 microglobulin, bone marrow aspiration, hepatitis B related test, HIV test, lumbar puncture, abdominal ultrasound.
Histologic type/diffuse large B-cell lymphoma, pathologic subtypes include primary mediastinal (thymic) large B-cell lymphoma, leaky B-cell lymphoma, and intravascular large B-cell lymphoma.
Typical immunophenotypes: CD20+ , CD45+, CD3-
Molecular genetic analysis tests: bcl-2, bcl-6, c-myc
Ann Arbor-Cotswolds staging.
I Invasion of one lymph node region or lymph-like structure (e.g., spleen, thymus, or Ring of Weymouth) (I); or one extra-lymph node organ or site (IE)
II Invasion of two or more lymph node areas on one side of the diaphragm (II) or localized continuation of an extra-lymph node organ or site combined with lymph node invasion in the ipsilateral area of the diaphragm (IIE) o
III Regional invasion of lymph nodes on both sides of the diaphragm (III), which may be combined with localized invasion of an extra-nodal organ or site across (IIIE); or combined with invasion of the spleen (IIIs); or invasion of an extra-nodal organ and spleen (IIIS+E).
IV is accompanied by extensive invasion of one or more distant extra-nodal organs.
The following definitions apply to each stage
A: No systemic symptoms
B: with systemic symptoms, defined as follows, as long as one of them is considered as B symptoms
1.Unexplained fever over 38℃ for 3 consecutive days
2.Unexplained weight loss >10% within 6 months
3, night sweats
E: continuous extra-nodal site invasion, or lymph node invasion of adjacent organs or tissues
S: Invasion of spleen
Treatment principles
Phase I and II induction therapy
l Non-megaloblastic type (<10 cm): ① Presence of adverse risk factors (elevated LDH, stage II, age >60 years, PS score ≥2): R-CHOP 6-8 courses ± local radiotherapy (IF 30-36 Gy) or R-CHOP ×3 courses + local radiotherapy (IF 30-36 Gy) ② Absence of adverse risk factors: R-CHOP ×3 courses + local radiotherapy ( IF 30-36Gy) or R-CHOP 6-8 courses.
l Giant mass (>10cm): R-CHOP 6-8 courses + local radiotherapy ( IF 30-36Gy) (Category 1).
Stage I and II follow-up (review of all positive results before radiotherapy evaluation)
Complete remission or CRu: follow-up after completion of the established course of treatment
Partial remission: completion of high-dose radiotherapy (40-45 Gy) or autologous stem cell transplantation or clinical trial, review at the end of treatment, follow-up for complete remission, others treated as relapse
No remission or disease progression: high-dose treatment or clinical trial.
Phase III and IV induction therapy
Low/low-intermediate risk (IPI0-1): R-CHOP 6-8 courses (Class 1)
Intermediate-high/high risk (IPI ≥ 2): Clinical trial (preferred) or R-CHOP 6-8 sessions (category 1)
Stage III, IV follow-up (review all positive results after 3-4 courses)
Complete remission or CRu: continue R-CHOP regimen until 6-8 courses of follow-up are achieved
Partial remission: continue R-CHOP regimen until 6-8 courses are reached or clinical trial, followed by review of all positive results, no remission or progression given as 2nd line therapy or stem cell transplant or clinical trial
No remission or disease progression: 2nd line therapy or stem cell transplantation or clinical trial with radiation therapy for patients not eligible for chemotherapy.
Recommended treatment options
First-line regimen: R-CHOP (class 1), R-ECHOP (class 2B)
Second-line treatment options: DHAP±R, ESHAP±R, GDP±R, ICE±R, MINE±R