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🫀 Interná medicína · 56
Lymfómy, Chronická lymfocytová leukémia
Definícia
- • Lymfómy sú zhubné klonové proliferácie lymfoidných buniek; delia sa na Hodgkinov lymfóm (pôvod z B-buniek, patognomické Reed-Sternbergove bunky) a heterogénnu skupinu non-Hodgkinových lymfómov. Chronická lymfocytová leukémia (CLL) je charakterizovaná progresívnou akumuláciou fenotypovo zrelých malígnych B-lymfocytov v periférnej krvi, kostnej dreni, slezine a lymfatických uzlinách.
Etiológia a patogenéza
- • Hodgkinov lymfóm je zriedkavý pred 10. rokom veku, najčastejší medzi 15. a 40. rokom; druhý vrchol výskytu je po 60. roku.
- • S Hodgkinovým lymfómom sa spája napr. predchádzajúca liečba fenytoínom, rádioterapia alebo chemoterapia a infekcia vírusom Epsteina-Barrovej (EBV) alebo HIV.
- • Non-Hodgkinove lymfómy: príčina je neznáma, pri časti prípadov sa predpokladá vírusová príčina (napr. HTLV-1, EBV, HBV, HCV, HIV, HHV-8); riziko zvyšuje aj infekcia Helicobacter pylori.
- • CLL je najčastejšou leukémiou v západnom svete; priemerný vek pacienta je 70 rokov, u detí je extrémne zriedkavá.
Klinický obraz
- • Hodgkinov lymfóm sa najčastejšie prejavuje nebolestivou krčnou lymfadenopatiou.
- • B-symptómy: horúčka, nočné potenie a nechcený úbytok hmotnosti > 10 % telesnej hmotnosti za posledných 6 mesiacov.
- • Non-Hodgkinove lymfómy: zväčšené mediastinálne a retroperitoneálne uzliny môžu utláčať okolité štruktúry, napr. hornú dutú žilu – dušnosť a edém tváre (syndróm hornej dutej žily).
- • CLL je v začiatkoch často asymptomatická alebo s nešpecifickými príznakmi (napr. únava, slabosť, úbytok hmotnosti, horúčka, nočné potenie); lymfadenopatiu má viac ako 50 % pacientov.
Diagnostika
- • Hodgkinov lymfóm: biopsia ukáže Reed-Sternbergove bunky (veľké dvojjadrové bunky) v charakteristicky heterogénnom infiltráte (histiocyty, lymfocyty, monocyty, plazmatické bunky, eozinofily).
- • Staging Hodgkinovho lymfómu: zobrazovacím vyšetrením voľby je kombinované FDG-PET/CT hrudníka, brucha a panvy.
- • CLL: na diagnózu upozorní absolútna lymfocytóza v periférnej krvi > 5000/µl (> 5 × 10⁹/l); klonalitu B-lymfocytov potvrdí prietoková cytometria periférnej krvi.
- • Imunofenotyp CLL: lymfocyty exprimujú CD5, CD19, CD20, CD23 a ľahký reťazec kappa alebo lambda.
Liečba
- • Hodgkinov lymfóm v limitovanom štádiu sa zvyčajne lieči skráteným režimom ABVD (doxorubicín, bleomycín, vinblastín, dakarbazín) s rádioterapiou alebo bez nej.
- • Agresívne B-bunkové lymfómy (napr. difúzny veľkobunkový B-lymfóm): štandardnou kombináciou je R-CHOP (rituximab, cyklofosfamid, doxorubicín, vinkristín, prednizón).
- • CLL: asymptomatickí pacienti a pacienti v skorom štádiu (napr. Rai < 3, Binet A alebo B) bez aktívneho ochorenia liečbu nepotrebujú – štandardom je sledovanie.
- • CLL – pri indikácii liečby sa v prvej línii dnes používajú najmä cielené lieky (napr. inhibítory BTK, inhibítor BCL-2 venetoklax); chemoimunoterapia (napr. FCR) bola predchádzajúcim štandardom.
Komplikácie a prognóza
- • CLL: hypogamaglobulinémia (až u dvoch tretín pacientov) so zvýšeným rizikom infekcií; sklon k autoimunitnej hemolytickej anémii (pozitívny priamy antiglobulínový test) a imunitnej trombocytopénii.
- • Richterov syndróm: asi 2 – 10 % prípadov CLL prechádza do difúzneho veľkobunkového B-lymfómu.
- • Neskoré následky liečby Hodgkinovho lymfómu: chemoterapia (napr. alkylačné látky, doxorubicín, etopozid) zvyšuje riziko leukémie medzi 3. a 10. rokom po liečbe.
- • Doxorubicín a ožiarenie mediastína zvyšujú riziko kardiomyopatie, koronárnej aterosklerózy a chlopňových chýb; bleomycín môže spôsobiť poškodenie pľúc (niekedy závažné, zriedka fatálne).
Kľúčové pojmy
| lymfóm | lymphoma |
| Hodgkinov lymfóm | lymphoma Hodgkin |
| non-Hodgkinov lymfóm | |
| Reed-Sternbergova bunka | |
| chronická lymfocytová leukémia | leucaemia lymphocytica chronica |
| lymfadenopatia | lymphadenopathia |
| B-symptómy | |
| difúzny veľkobunkový B-lymfóm | |
| Richterov syndróm | |
| hypogamaglobulinémia | hypogammaglobulinaemia |
| autoimunitná hemolytická anémia | anaemia haemolytica autoimmunis |
| prietoková cytometria |
📎 Zdroje:
- Non-Hodgkin Lymphomas – MSD Manual Professional Edition (Definition)
Non-Hodgkin lymphomas are a heterogeneous group of disorders involving malignant, monoclonal proliferation of lymphoid cells in lymphoreticular sites
- Hodgkin Lymphoma – MSD Manual Professional Edition (Pathophysiology)
Hodgkin lymphoma results from the clonal transformation of cells of B-cell origin, giving rise to pathognomic binucleated Reed-Sternberg cells.
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Definition)
Chronic lymphocytic leukemia (CLL) is characterized by progressive accumulation of phenotypically mature malignant B lymphocytes. Primary sites of disease include peripheral blood, bone marrow, spleen, and lymph nodes.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Epidemiology)
Hodgkin lymphoma is rare before age 10 years and is most common between ages 15 and 40 years; a second peak occurs in people > 60 years.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Etiology)
Environmental associations connected with Hodgkin lymphoma include history of treatment with phenytoin, radiation therapy, or chemotherapy and infection with Epstein-Barr virus (EBV) or HIV.
- Non-Hodgkin Lymphomas – MSD Manual Professional Edition (Etiology)
substantial evidence suggests a viral cause (eg, human T-cell leukemia-lymphoma virus, Epstein-Barr virus, hepatitis B virus, hepatitis C virus, HIV, human herpesvirus 8) in some cases. Helicobacter pylori infection also increases lymphoma risk.
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Epidemiology)
Chronic lymphocytic leukemia is the most common type of leukemia in the Western world.
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Epidemiology)
The average age of a patient with CLL is 70 years; CLL is extremely rare in children.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Symptoms and Signs)
Most patients with Hodgkin lymphoma present with painless cervical adenopathy.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Symptoms and Signs)
Systemic symptoms include fever, night sweats, and loss of appetite resulting in unintentional weight loss (> 10% of body weight in previous 6 months), which are referred to as "B symptoms."
- Non-Hodgkin Lymphomas – MSD Manual Professional Edition (Symptoms and Signs)
In some patients, enlarged mediastinal and retroperitoneal nodes press on nearby structures, leading to symptoms. The most important of these are Compression of the superior vena cava (SVC): Shortness of breath and facial edema (SVC syndrome)
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Symptoms and Signs)
Patients are often asymptomatic early on, with insidious onset of nonspecific symptoms (eg, fatigue, weakness, anorexia, weight loss, fever, and/or night sweats) that may prompt evaluation. More than 50% of patients have lymphadenopathy.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Diagnosis)
Biopsy reveals Reed-Sternberg cells (large, binucleated cells) in a characteristically heterogeneous cellular infiltrate, consisting of histiocytes, lymphocytes, monocytes, plasma cells, and eosinophils.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Diagnosis)
A combined fluorodeoxyglucose (FDG)-PET/CT scan of the chest, abdomen, and pelvis is the imaging study of choice for staging Hodgkin lymphoma
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Diagnosis)
The diagnosis of chronic lymphocytic leukemia is first suspected when an absolute peripheral lymphocytosis of > 5000/mcL (> 5 × 109/L) is found. Peripheral blood flow cytometry can confirm clonality in circulating B cells.
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Diagnosis)
The circulating lymphocytes should express CD5, CD19, CD20, CD23, and kappa or lambda light chains.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Treatment)
Limited-stage disease is generally treated with an abbreviated chemotherapy regimen of doxorubicin (Adriamycin), bleomycin, vinblastine, and dacarbazine (ABVD) with or without radiation therapy.
- Non-Hodgkin Lymphomas – MSD Manual Professional Edition (Treatment)
In patients with aggressive B-cell lymphomas (eg, diffuse large B cell), the standard combination is rituximab plus cyclophosphamide, hydroxydaunorubicin (doxorubicin), vincristine, and prednisone (R-CHOP).
- Chronic Lymphocytic Leukemia – StatPearls – NCBI Bookshelf (Treatment / Management)
For CLL patients who are asymptomatic or have early-stage disease (e.g., Rai stage <3, Binet stage A or B), observation is the standard of care, as they do not require treatment.
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Summary)
first-line regimens may include targeted agents such as inhibitors of Bruton tyrosine kinase (BTK) and B-cell lymphoma-2 (BCL-2)
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Pathophysiology)
Hypogammaglobulinemia can develop in up to two-thirds of patients, increasing risk for infectious complications. Patients have increased susceptibility to autoimmune hemolytic anemia (with a positive direct antiglobulin test) and immune thrombocytopenia.
- Chronic Lymphocytic Leukemia (CLL) – MSD Manual Professional Edition (Pathophysiology)
Approximately 2 to 10% of CLL cases develop into diffuse large B-cell lymphoma (called Richter syndrome)
- Hodgkin Lymphoma – MSD Manual Professional Edition (Complications of treatment)
Chemotherapy, particularly with drugs such as the alkylating agents (mechlorethamine, cyclophosphamide, procarbazine), doxorubicin, and etoposide, increase the risk of leukemia between years 3 and 10 post-therapy.
- Hodgkin Lymphoma – MSD Manual Professional Edition (Complications of treatment)
Doxorubicin as well as mediastinal radiation increases the risk of cardiomyopathy, coronary atherosclerosis and valvular heart disease. Bleomycin can induce lung injury, which can be severe and rarely fatal.
- MSD Manual Professional – Chronic Lymphocytic Leukemia (Treatment)
Studies have suggested that targeted therapy is as efficacious if not superior to upfront chemoimmunotherapy for most patients
- MSD Manual Professional – Chronic Lymphocytic Leukemia (Treatment)
The combination of fludarabine, cyclophosphamide, and rituximab (FCR) was the prior standard of care for upfront treatment in most medically fit patients
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