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Pàgina nova, amb el contingut: «El '''limfoma de Hodgkin''', també conegut com a '''malaltia de Hosgkin''', és una mena de linfoma, un càncer que s'origina a un tipus específic de glòbuls blancs anomenat limfòcits, en què cèl·lules gegants anomenades de Reed-Sternberg són presents en els ganglis limfàtics de la persona afectada.<ref name="NCI2016AdPt">{{cite web|title=Adult Hodgkin Lymphoma Treatment (PDQ)–Patient Version|url=htt...».
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Revisió del 22:21, 22 ago 2021

El limfoma de Hodgkin, també conegut com a malaltia de Hosgkin, és una mena de linfoma, un càncer que s'origina a un tipus específic de glòbuls blancs anomenat limfòcits, en què cèl·lules gegants anomenades de Reed-Sternberg són presents en els ganglis limfàtics de la persona afectada.[1][2]

Un limfoma és un tipus de càncer que s'origina en el teixit limfàtic. Aquest teixit comprèn els ganglis limfàtics i els òrgans relacionats, que formen part del sistema immunitari i del sistema productor de sang del cos. També es troben a moltes altres parts del cos, per exemple dins del tòrax, l'abdomen i la pelvis. A causa que el teixit limfàtic es troba a nombroses parts del cos, la malaltia de Hodgkin pot originar-se en gairebé a qualsevol part, però en general s'origina als ganglis limfàtics de la part superior del cos, sent el tòrax, el coll i les aixelles les àrees més comunes. Aquest tipus de càncer produeix un engrandiment del teixit limfàtic, la qual cosa pot ocasionar pressió sobre algunes estructures importants. La forma principal de propagació de la malaltia de Hodgkin és a través dels vasos limfàtics a altres ganglis limfàtics. La majoria de vegades aquesta malaltia es propaga als ganglis limfàtics propers al cos i no als distants. Poques vegades aconsegueix passar als vasos sanguinis i pot estendre's a gairebé qualsevol altra part del cos, incloent-hi el fetge i els pulmons.Els símptomes poden incloure febre, sudoració nocturna, i pèrdua de pes.[1] Sovint, s'hi troba un creixement dels ganglis limfàtics al coll, sota el braç, o en l'engonal.[1] També apareix fatiga i pruïja.[1][3]

Existeixen nombroses raons per les quals els ganglis limfàtics poden augmentar de mida. Encara que això pot ser conseqüència de la malaltia de Hodgkin, amb molta més freqüència és un indicador que el cos està combatent una infecció.

Hi ha dos grans tipus de limfoma de Hodgkin: limfoma de Hodgkin clàssic (cHL en anglés) i el limfoma de Hodgkin nodular amb limfòcits predominants. [4] Aproximadament la meitat dels casos de limfoma de Hodgkin són arran de la infecció amb el virus d'Epstein-Barr (VEB) i generalment causen la forma clàssica.[5][6] Altres factors de risc inclouen una història familiar de la condició i tindre una infecció per VIH.[1][5] El diagnòstic s'assoleix a través de confirmar la presència del càncer i identificar les cèlules de Reed-Sternberg en una biòpsia d'un gangli limfàtic.[1] Les formes positives pel virus d'Epstein-Barr són classificades com una forma de malaltia limfoproliferativa limfoide associada al virus d'Epstein Barr.[7]

El limfoma de Hodgkin es pot tractar amb quimioteràpia, radioteràpia, i un transplantament de medul·la òssia.[8] El tipus de tractament sovint depén de com d'avançat es trobe el càncer i si presenta característiques favorables o no.[8] En els estadis més primerencs, sovint és possible la curació del càncer.[9] Als Estats Units, el 88% de la gent diagnosticada amb limfoma de Hodgkin té una supervivència superior als cinc anys o superior.[4] Per als menors de 20 anys, la taxa de supervivència és del 97%.[10] A Catalunya, té una taxa de supervivència a cinc anys per a homes al voltant del 85% i per a dones al voltant del 87%.[11] No obstant això, la radiació i la quimioteràpia augmenten el risc de patir un altre càncer, malaltia cardiovascular, o malaltia pulmonar durant les següents dècades.[9]

El 2015, al voltant de 574.000 persones arreu del món patien limfoma de Hodgkin, i d'elles 23.900 (un 4,2%) van morir.[12][13] Als Estats Units, un 0,2% de la població es troba afectada en algun moment de la seua vida.[4] És un tipus de limfoma maligne, encara que molt menys freqüent que el limfoma no hodgkinià. L'edat més comú de diagnòstic és entre els 20 i 40 anys.[4] Rep el seu nom del metge anglés Thomas Hodgkin, el primer en descriure la condició en 1832.[9][14]

Símptomes

Aquesta mena de limfoma es pot presentar amb aquests símptomes:

  • Limfadenopatia: el símptoma que es troba més freqüentment és el creixement no dolorós d'un o diversos ganglis limfàtics.[15] Els ganglis acostumen a tindre un tacte dur i inflamat quan s'examinen. Els ganglis del coll, aixelles i engonals són els més involucrats (aproximadament un 80-90% dels casos).[15] Els ganglis del pit es troben afectats sovint, i es poden observar en una radiografia de tòrax.[15]
  • Símptomes sistèmics: un terç de les persones amb limfoma de Hodgkin poden presentar els següents símptomes:[16]
    • Pruïja
    • Hiperhidrosi nocturna
    • Pèrdua de pes sense explicar d'almenys un 10% de la massa corporal total de la persona en sis mesos o menys
    • Febrícula
    • Fatiga.
    • Aquests símptomes es coneixen com a símptomes B, i la presència d'aquests símptomes indica l'estadiatge de la persona.
  • Esplenomegàlia: sovint podem trobar l'engrandiment de la melsa en el limfoma de Hodgkin. Aquest creixement, però, rara volta és massiu, i la mida de la melsa pot fluctuar durant el tractament.[15]
  • Hepatomegàlia: encara que de forma infreqüent, es pot trobar l'engrandiment del fetge.[15]
  • Hepatosplenomegàlia: a vegades es troba hepatomegàlia i esplenomegàlia en un mateix individu.
  • Dolor després de consumir alcohol: clàssicament, els ganglis afectats fan mal després de consumir alcohol, però aquest fenomen és molt rar,[17] ocorrent en només d'un dos a tres percent de persones amb limfoma de Hodgkin,[18] per això té una baixa sensibilitat. D'altra banda, té un valor predictiu positiu prou alt per a ser considerar un signe patognomònic del limfoma de Hodgkin.[18] El dolor típicament apareix en pocs minuts d'ingerir alcohol, i se sent als voltants dels ganglis limfàtics afectats.[18] Aquest dolor s'ha descrit com a agut i punxant o sord i general.[18]
  • Mal d'esquena: un mal d'esquena inespecífic (un dolor que no es pot localitzar o determinar una causa a través de l'examinació física o amb proves d'imatge) s'ha descrit en alguns casos de limfoma de Hodgkin. Sovint es troba afectada l'esquena inferior.[19]
  • Febre cíclica: algunes persones poden presentar una febre cícilica i d'alta temperatura coneguda com a febre de Pel-Ebstein,[20] o simplement "febre P-E". No obstant això hi ha un debat sobre la validesa d'aquest signe.[21]
  • Una síndrome nefròtica pot sorgir en individus amb limfoma de Hodgkin, sovint causada per una malaltia de canvis mínims.[22]
  • Poden presentar obstrucció de les vies respiratòries, efusió pleural/del pericardi, disfunció hepatocel·lular, infiltració de la medul·la òssia.

Diagnòstic

El limfoma de Hodgkin s'ha de distingir de causes no malignes del creixement dels ganglis limfàtics (com diverses infeccions) i altres tipus de càncer. El diagnòstic definitiu es fa a través de la biòpsia d'un gangli limfàtic (usualment amb una exició i la posterior anàlisi de microscopi). L'hemograma pot servir per a jutjar la funció dels òrgans principals i per a comprovar la seguretat de la quimioteràpia. El PET-TAC s'utilitza per a trobar xicotets dipòsits que no es mostren en els TAC.

Tipus

Hi ha dos tipus principals de limfoma de Hodgkin: el clàssic i el limfoma de Hodgkin nodular amb limfòcits predominants. La prevalència del clàssic i del nodular són del 90% i el 10% respectivament.[23][24] La morfologia, fenotip, característiques moculars, i presentació i compartament clínic d'aquests dos grups són, aleshores, diferent.[25]

Clàssic

El limfoma de Hodgkin clàssic es pot sublassificar en quatre subtipus patològics basats en la morfologia de les cèl·lules de Reed-Sternberg i la composició de l'infiltrat reactiu de la cèl·lula que s'ha pogut observar en la biòpsia del gangli limfàtic (és a dir, quines cèl·lules acompanyes a la cèlula R-S).

Nom Descripció
Limfoma de Hodgkin amb esclerosi nodular És el subtipus més comú i es composa de grans nòduls tumorals mostrant cèl·lules R-S disperses en un rerefons de limfòcits, eosinòfils i plasmòcits reactius amb graus variables de fibrosi del colàgen o esclerosi.
Subtipus de cel·lularitat mixta És un subtipus comú i es caracteritza per tindre nombroses cèl·lules RS clàssiques barrejades amb nombres cèl·lules inflamatòries reactives com limfòcits, histiòcits, eosinòfils, i plasmòcits sense esclerosi. Aquest tipus s'associa freqüentment a la infecció amb el virus d'Epstein-Barr i es pot confondre amb l'anomenada fase "cel·lular" o temprana del subtipus d'esclerosi nodular. Aquesta mena de limfoma de Hodgkin es troba sovint en pacients immunodeprimits.
Ric en limfòcits És un subtipus rar que mostra moltes característiques el podrien confondre amb el limfoma no hodgkinià de cèl·lules B amb limfòcits nodulars predominants. Aquesta forma té el millor pronòstic.
Baix en limfòcits És un subtipus rar, composat de grans quantitats de cèl·lules RS pleomòrfiques amb només uns quants limfòcits reactius que es pot confondre amb limfoma difús de cèl·lules B grans. Molts d'aquests casos es classificarien en l'actualitat dins dels limfoma anaplàstic de cèl·lules gegants.[26]
 
Lymph node biopsy showing Hodgkin lymphoma, mixed-cellularity type
 
CT image of a 46-year-old person with Hodgkin lymphoma, image at neck height. On the left side of the person's neck enlarged lymph nodes are visible (marked in red).

Encara que no s'expressen en totes les cèl·lules els marcadors tradicionals dels limfòcits B (com el CD20),[26] les cèl·lules de Reed-Sternberg sovint són de tipus B.[27][28] Encara que sovint es classifica al limfoma de Hodgkin amb altres càncers de cèl·lula B, s'expressen alguns marcadors de limfòcits T (com a CD2 and CD4) de forma ocasional.[29] No obstant això, açò pot ser arran

Hodgkin cells produce interleukin-21 (IL-21), which was once thought to be exclusive to T-cells. This feature may explain the behavior of classic Hodgkin lymphoma, including clusters of other immune cells gathered around HL cells (infiltrate) in cultures.[30]

Nodular lymphocyte predominant

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is another subtype of Hodgkin lymphoma distinct from Classic Hodgkin lymphoma and is characterized by the presence of popcorn cells which express CD20.[24][31] Due to these differences, among others, NLPHL is often treated differently from Classic Hodgkin lymphoma, including using rituximab in combination with AVBD chemotherapy, though individual cases vary and clinical trials are ongoing.[24]

Staging

The staging is the same for both Hodgkin and non-Hodgkin lymphomas.

After Hodgkin lymphoma is diagnosed, a person will be staged: that is, they will undergo a series of tests and procedures that will determine what areas of the body are affected. These procedures may include documentation of their histology, a physical examination, blood tests, chest X-ray radiographs, computed tomography (CT)/Positron emission tomography (PET)/magnetic resonance imaging (MRI) scans of the chest, abdomen and pelvis, and usually a bone marrow biopsy. Positron emission tomography (PET) scan is now used instead of the gallium scan for staging. On the PET scan, sites involved with lymphoma light up very brightly enabling accurate and reproducible imaging.[32] In the past, a lymphangiogram or surgical laparotomy (which involves opening the abdominal cavity and visually inspecting for tumors) were performed. Lymphangiograms or laparotomies are very rarely performed, having been supplanted by improvements in imaging with the CT scan and PET scan.[33]

On the basis of this staging, the person will be classified according to a staging classification (the Ann Arbor staging classification scheme is a common one):

  • Stage I is involvement of a single lymph node region (I) (mostly the cervical region) or single extralymphatic site (Ie);
  • Stage II is involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and a contiguous extralymphatic site (IIe);
  • Stage III is involvement of lymph node regions on both sides of the diaphragm, which may include the spleen (IIIs) or limited contiguous extralymphatic organ or site (IIIe, IIIes);
  • Stage IV is disseminated involvement of one or more extralymphatic organs.

The absence of systemic symptoms is signified by adding "A" to the stage; the presence of systemic symptoms is signified by adding "B" to the stage. For localised extranodal extension from mass of nodes that does not advance the stage, subscript "E" is added. Splenic involvement is signified by adding "S" to the stage. The inclusion of "bulky disease" is signified by "X".

Pathology

Macroscopy

Affected lymph nodes (most often, laterocervical lymph nodes) are enlarged, but their shape is preserved because the capsule is not invaded. Usually, the cut surface is white-grey and uniform; in some histological subtypes (e.g. nodular sclerosis) a nodular aspect may appear.

A fibrin ring granuloma may be seen.

Microscopy
 
Micrograph of a classic Reed–Sternberg cell
 
Micrograph showing a "popcorn cell", the Reed–Sternberg cell variant seen in nodular lymphocyte predominant Hodgkin lymphoma. H&E stain

Microscopic examination of the lymph node biopsy reveals complete or partial effacement of the lymph node architecture by scattered large malignant cells known as Reed-Sternberg cells (RSC) (typical and variants) admixed within a reactive cell infiltrate composed of variable proportions of lymphocytes, histiocytes, eosinophils, and plasma cells. The Reed–Sternberg cells are identified as large often bi-nucleated cells with prominent nucleoli and an unusual CD45-, CD30+, CD15+/- immunophenotype. In approximately 50% of cases, the Reed–Sternberg cells are infected by the Epstein–Barr virus.[34]

Characteristics of classic Reed–Sternberg cells include large size (20–50 micrometres), abundant, amphophilic, finely granular/homogeneous cytoplasm; two mirror-image nuclei (owl eyes) each with an eosinophilic nucleolus and a thick nuclear membrane (chromatin is distributed close to the nuclear membrane). Almost all of these cells have an increased copy number of chromosome 9p/9p24.1.[35]

Variants:

  • Hodgkin cell (atypical mononuclear RSC) is a variant of RS cell, which has the same characteristics but is mononucleated.
  • Lacunar RSC is large, with a single hyperlobulated nucleus, multiple, small nucleoli and eosinophilic cytoplasm which is retracted around the nucleus, creating an empty space ("lacunae").
  • Pleomorphic RSC has multiple irregular nuclei.
  • "Popcorn" RSC (lympho-histiocytic variant) is a small cell, with a very lobulated nucleus, small nucleoli.
  • "Mummy" RSC has a compact nucleus with no nucleolus and basophilic cytoplasm.

Hodgkin lymphoma can be sub-classified by histological type. The cell histology in Hodgkin lymphoma is not as important as it is in non-Hodgkin lymphoma: the treatment and prognosis in classic Hodgkin lymphoma usually depends on the stage of disease rather than the histotype.[36]

Management

The current approach for treatment aims to reduce the acute and long-term toxicities associated with Hodgkin lymphoma (e.g. cardiac damage and secondary cancers) and increase overall survival.[37]

People with early stage disease (IA or IIA) are effectively treated with radiation therapy or chemotherapy. The choice of treatment depends on the age, sex, bulk and the histological subtype of the disease.[38] Adding localised radiation therapy after the chemotherapy regimen may provide a longer progression-free survival compared with chemotherapy treatment alone.[39] People with later disease (III, IVA, or IVB) are treated with combination chemotherapy alone. People of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy.[cal citació]

MOPP ABVD Stanford V BEACOPP
The original treatment for Hodgkin's was MOPP. The abbreviation stands for the four drugs Mustargen (also known as chlormethine), Oncovin (also known as vincristine), Prednisone and Procarbazine (also known as Matulane). The treatment is usually administered in four week cycles, often for six cycles. MSD and VCR are administered intravenously, while procarbazine and prednisone are pills taken orally. MOPP was the first combination chemotherapy brought in that achieved a high success rate. It was developed at the National Cancer Institute in the 1960s by a team that included Vincent DeVita Jr.

Although no longer the most effective combination, MOPP is still used after relapse or where the person has certain allergies or lung or heart problems which prevents the use of another regimen.

Currently, the ABVD chemotherapy regimen is the standard treatment of Hodgkin's disease in the US. The abbreviation stands for the four drugs Adriamycin, bleomycin, vinblastine, and dacarbazine. Developed in Italy in the 1970s, the ABVD treatment typically takes between six and eight months, although longer treatments may be required. The newer Stanford V regimen is typically only half as long as the ABVD but involves a more intensive chemotherapy schedule and incorporates radiation therapy. In a randomised controlled study in Italy, Stanford V was inferior to ABVD;[40] however, this study has been heavily criticized due to its incorrect administration of radiotherapy, diverging from the original Stanford V protocol.[41] BEACOPP is a form of treatment for stages > II mainly used in Europe. The cure rate with the BEACOPP esc. regimen is approximately 10–15% higher than with standard ABVD in advanced stages. This was shown in a paper in The New England Journal of Medicine (Diehl et al.), but US physicians still favor ABVD, maybe because some physicians think that BEACOPP induces more secondary leukemia. However, this seems negligible compared to the higher cure rates. BEACOPP is more expensive because of the requirement for concurrent treatment with GCSF to increase production of white blood cells. Currently, the German Hodgkin Study Group tests 8 cycles (8x) BEACOPP esc vs. 6x BEACOPP esc vs. 8x BEACOPP-14 baseline (HD15-trial).[42]
Chlormethine Doxorubicin Doxorubicin Doxorubicin
Oncovin Bleomycin Bleomycin Bleomycin
Prednisone Vinblastine Vinblastine, Vincristine Vincristine
Procarbazine Dacarbazine Chlormethine Cyclophosphamide, Procarbazine
Etoposide Etoposide
Prednisone Prednisone

The common non-Hodgkin treatment, rituximab (which is a monoclonal antibody against CD20) is not routinely used to treat Hodgkin lymphoma due to the lack of CD20 surface antigens in most cases. The use of rituximab in Hodgkin lymphoma, including the lymphocyte predominant subtype has been recently reviewed.[43] The evidence is very uncertain about the effect of Nivolumab for patients with a Hodgkin’s lymphoma e.g. on the overall survival.

Although increased age is an adverse risk factor for Hodgkin lymphoma, in general elderly people without major comorbidities are sufficiently fit to tolerate standard therapy, and have a treatment outcome comparable to that of younger people. However, the disease is a different entity in older people and different considerations enter into treatment decisions.[44]

For Hodgkin lymphomas, radiation oncologists typically use external beam radiation therapy (sometimes shortened to EBRT or XRT). Radiation oncologists deliver external beam radiation therapy to the lymphoma from a machine called a linear accelerator which produces high energy X-rays and electrons. People usually describe treatments as painless and similar to getting an X-ray. Treatments last less than 30 minutes each.

For lymphomas, there are a few different ways radiation oncologists target the cancer cells. Involved site radiation is when the radiation oncologists give radiation only to those parts of the person's body known to have the cancer.[45] Very often, this is combined with chemotherapy. Radiation therapy directed above the diaphragm to the neck, chest or underarms is called mantle field radiation. Radiation to below the diaphragm to the abdomen, spleen or pelvis is called inverted-Y field radiation. Total nodal irradiation is when the therapist gives radiation to all the lymph nodes in the body to destroy cells that may have spread.[46]

Adverse effects

The high cure rates and long survival of many people with Hodgkin lymphoma has led to a high concern with late adverse effects of treatment, including cardiovascular disease and second malignancies such as acute leukemias, lymphomas, and solid tumors within the radiation therapy field. Most people with early-stage disease are now treated with abbreviated chemotherapy and involved site radiation therapy rather than with radiation therapy alone. Clinical research strategies are exploring reduction of the duration of chemotherapy and dose and volume of radiation therapy in an attempt to reduce late morbidity and mortality of treatment while maintaining high cure rates. Hospitals are also treating those who respond quickly to chemotherapy with no radiation.

In childhood cases of Hodgkin lymphoma, long-term endocrine adverse effects are a major concern, mainly gonadal dysfunction and growth retardation. Gonadal dysfunction seems to be the most severe endocrine long-term effect, especially after treatment with alkylating agents or pelvic radiotherapy.[47]

It is possible that patients undergoing a chemotherapy need a platelet transfusion.[48][49] If a stem cell transplantation is necessary for the treatment of a relapse, graft-versus-host diseases might occur.[50]

Supportive treatment

Adding physical exercises to the standard treatment for adult patients with haematological malignancies like Hodgkin lymphoma may result in little to no difference in the mortality, the quality of life and the physical functioning. These exercises may result in a slight reduction in depression. Furthermore, aerobic physical exercises probably reduce fatigue. The evidence is very uncertain about the effect on anxiety and serious adverse events.[51] 

Prognosis

Treatment of Hodgkin's disease has been improving over the past few decades. Recent trials that have made use of new types of chemotherapy have indicated higher survival rates than have previously been seen. In one recent European trial, the 5-year survival rate for those people with a favorable prognosis (FFP) was 98%, while that for people with worse outlooks was at least 85%.[52]

In 1998, an international effort[53] identified seven prognostic factors that accurately predict the success rate of conventional treatment in people with locally extensive or advanced-stage Hodgkin lymphoma. Freedom from progression (FFP) at 5 years was directly related to the number of factors present in a person. The 5-year FFP for people with zero factors is 84%. Each additional factor lowers the 5-year FFP rate by 7%, such that the 5-year FFP for a person with 5 or more factors is 42%.

The adverse prognostic factors identified in the international study are:

Other studies have reported the following to be the most important adverse prognostic factors: mixed-cellularity or lymphocyte-depleted histologies, male sex, large number of involved nodal sites, advanced stage, age of 40 years or more, the presence of B symptoms, high erythrocyte sedimentation rate, and bulky disease (widening of the mediastinum by more than one third, or the presence of a nodal mass measuring more than 10 cm in any dimension.)

More recently, the use of positron emission tomography (PET) early after commencing chemotherapy has demonstrated to have powerful prognostic ability.[54] This enables assessment of an individual's response to chemotherapy as the PET activity switches off rapidly in people who are responding. In this study,[54] after two cycles of ABVD chemotherapy, 83% of people were free of disease at 3 years if they had a negative PET versus only 28% in those with positive PET scans. This prognostic method improves on FFP estimates based on the seven conventional factors. Several trials are underway to see if PET-based risk adapted response can be used to improve a person's outcomes by changing chemotherapy early in people who are not responding.

The evidence is very uncertain about the effect of negative (= good prognosis) or positive (= bad prognosis) interim PET scan results for patients with a Hodgkin’s lymphoma on the progression-free survival. Negative interim PET scan results may result in an increase in progression-free survival compared if the adjusted result was measured. Negative interim PET scan results probably result in a large increase in the overall survival compared to those with a positive interim PET scan result,[37]

Epidemiology

 
Age-standardized death from lymphomas and multiple myeloma per 100,000 inhabitants in 2004[55]
  no data
  less than 1.8
  1.8–3.6
  3.6–5.4
  5.4–7.2
  7.2–9
  9–10.8
  10.8–12.6
  12.6–14.4
  14.4–16.2
  16.2–18
  18–19.8
  more than 19.8

Unlike some other lymphomas, whose number of new cases per year increases with age, Hodgkin lymphoma has a bimodal curve for the number of cases; that is, it occurs most frequently in two separate age groups, the first being young adulthood (age 15–35) and the second being in those over 55 years old although these peaks may vary slightly with nationality.[56] Overall, it is more common in males, except for the nodular sclerosis variant, which is slightly more common in females. The annual number of cases of Hodgkin lymphoma is 2.7 per 100,000 per persons per year, and the disease accounts for slightly less than 1% of all cancers worldwide.[57]

In 2010, globally it resulted in about 18,000 deaths down from 19,000 in 1990.[58] In 2012, there were an estimated 65,950 cases and 25,469 deaths from Hodgkin lymphoma worldwide, with 28,852 and 37,098 cases occurring in developed and developing countries, respectively.[59] However, the age-standardized rates were higher in developed regions, with the greatest rates in the Americas (1.5 per 100,000), East Mediterranean Region (1.5 per 100,000), and Europe (2.0 per 100,000).[59] The East Mediterranean Region also has the highest age-standardized mortality rate of 1.0 per 100,000, which is mainly attributed to lifestyle and environmental risk factors associated with transitional economies such as smoking, obesity, physical inactivity, and reproductive behaviors, as well as availability of diagnostic practices and awareness of the disease.[59]

The number of cases of Hodgkin lymphoma is increased in people with HIV infection.[60] In contrast to many other lymphomas associated with HIV infection it occurs most commonly in people with higher CD4 T cell counts.

Canada

Hodgkin lymphoma accounts for 0.6% of all male cancer cases, and 0.4% of all female cancer cases in Canada. In 2017, approximately 990 Canadians will be diagnosed with Hodgkin lymphoma, and 140 will die of the disease.[61]

UK

Hodgkin lymphoma accounts for less than 1% of all cancer cases and deaths in the UK. Around 1,800 people were diagnosed with the disease in 2011, and around 330 people died in 2012.[62]

United States

In 2016, there were 8,389 new cases and 1,000 mortalities attributed to Hodgkin Lymphoma, a decrease from the 8,625 new cases and 1,120 mortalities in 2015.[63] As of January 1, 2016, the 5-year limited duration prevalence of Hodgkin Lymphoma was 37,513 representing 0.71% of all diagnosed cancers in the U.S.[63]

History

 
Photograph of Hodgkin's disease from a 1938 medical textbook

Hodgkin lymphoma was first described in an 1832 report by Thomas Hodgkin, although Hodgkin noted that perhaps an earlier reference to the condition was provided by Marcello Malpighi in 1666.[64][14] While occupied as museum curator at Guy's Hospital, London, Hodgkin studied seven people with painless lymph node enlargement. Of the seven cases, two were under the care of Richard Bright, one was of Thomas Addison, and one was of Robert Carswell.[64] Carswell's report of the seventh case was accompanied by numerous illustrations that aided early descriptions of the disease.[65]

Hodgkin's report on the seven cases, entitled "On some morbid appearances of the absorbent glands and spleen", was presented to the Medical and Chirurgical Society of London in January 1832 and was subsequently published in the society's journal, Medical-Chirurgical Society Transactions.[64] Hodgkin's paper went largely unnoticed, however, even though Bright highlighted it in an 1838 publication.[64] Indeed, Hodgkin himself did not view his contribution as particularly significant.[66]

In 1856, Samuel Wilks independently reported on a series of patients with the same disease that Hodgkin had previously described.[66] Wilks, a successor to Hodgkin at Guy's Hospital, was unaware of Hodgkin's prior work on the subject. Bright informed Wilks of Hodgkin's contribution and in 1865, Wilks published a second paper, entitled "Cases of enlargement of the lymphatic glands and spleen", in which he named the illness "Hodgkin's disease" in honor of his predecessor.[66]

Theodor Langhans and WS Greenfield first described the microscopic characteristics of Hodgkin lymphoma in 1872 and 1878, respectively.[64] In 1898 and 1902, respectively, Carl Sternberg and Dorothy Reed independently described the cytogenetic features of the malignant cells of Hodgkin lymphoma, now called Reed–Sternberg cells.[64]

Tissue specimens from Hodgkin's seven cases were preserved at Guy's Hospital. Nearly 100 years after Hodgkin's initial publication, histopathologic reexamination confirmed Hodgkin lymphoma in only three of seven of these people.[66] The remaining cases included non-Hodgkin lymphoma, tuberculosis, and syphilis.[66]

Hodgkin lymphoma was one of the first cancers to be treated successfully with radiation therapy and, later, it was one of the first to be treated by combination chemotherapy.

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Further reading

  • Charlotte DeCroes Jacobs. Henry Kaplan and the Story of Hodgkin's Disease (Stanford University Press; 2010) 456 pages; combines a biography of the American radiation oncologist (1918–84) with a history of the lymphatic cancer whose treatment he helped to transform.