Hodgkin Lymphoma

Alternative Names

  • HL
  • Hodgkin Disease
  • HD
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WHO-ICD-10 version:2010

Neoplasms

Malignant neoplasms

OMIM Number

236000

Mode of Inheritance

Autosomal recessive

Description

Hodgkin Disease (HD) is a malignant tumor affecting 5.5% of all pediatric cancers. It is characterized by pleomorphic lymphocytic and histiocytic infiltrate with multinucleated Reed-Sternberg cells (RS). The RS cells are clones of B origin which are located in the lymphoid germinal centres. It consists only 2% of all HD affected tissues, whereas the remaining cells include lymphocytes, plasma cells, neutrophils, eosinophils, and histiocytes. HD is histologically divided into five types. Four of these types are referred to as classic Hodgkin disease which includes: nodular sclerosis, mixed-cellularity, lymphocyte-depleted, and lymphocyte-rich. The fifth type (non-classical) is nodular lymphocyte predominant Hodgkin disease. About two thirds of pediatric patients are present with the nodular sclerosis type at diagnosis. The primary nodal site of HD is situated above the diaphragm in two third of patients. The usual clinical presentation consists of painless cervical or supraclavicular adenopathy. Splenomegaly and hepatomegaly often indicate advanced disease. The systemic symptoms of HD are typical of B symptoms, including fever, weight loss of 10%, and night sweats. These symtoms are expressed due to the secretion of at least twelve cytokines, including interleukin-1 (IL-1), IL-6 and tumr necrosis factor from the RS cells. In addition, the malignant RS cells are known to constitutively express high levels of activated nuclear factor kappa B (NFKB), which plays an important role in their survival. Also, the RS cells consistently express the CD30 and CD15 antigens, which are serving as malignant markers in the classical types. However, the RS cells are either infrequent or absent in nodular lymphocyte predominant type.

The etiology of HD is unknown. However, Epstein-Barr virus (EBV) has been reported to involve in the etiology of HD. In addition, other studies suggest other environmental factors such as exposure to pesticides or tonillectomy may also play a role.

Unlike most malignant tumors, HD is curable and the overall survival is more than 90% due to the pregressive advances in new diagnostic methods (CT scan, MR and Gallium scan) and treatment. Currently, all children receive combind chemotherapy with low dose irradiation (1500-2000 cGy) solely in the initially involved area instead of high doess (3600-4000 cGy).

Molecular Genetics

HD has shown familial occurrence in approximately 1% of HD patients. It was found that HLA-DP alleles are common in HD. In addition, several studies found mutations in the nuclear factor kappa B inhibitors genes in most HD cases.

Epidemiology in the Arab World

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Other Reports

Bahrain

Hamadeh et al. (1981) compared 125 cases of leukemia, Hodgkin's disease, and other lymphomas with 125 match controls for space, and time and space clustering in the State of Bahrain. These cases and controls were identified from hospital, pathology and death registries over a period of ten years. Thirty-one percent of the cases living in cities had "effective contact' with at least another case compared to 8.7 percent of the city controls, at a distance of "effective contact' equal to or less than 100 meters. Hamadeh et al. (1981) did not observe any differences in the number of "effective contacts" between the cases and controls residing in the villages. Combined data from the villages and urban areas were analyzed using two different methods. Also, Hamadeh et al. (1981) could not report any significant clustering.

Shome et al. (2004) conducted a retrospective hospital-based study to determine the clinicopathologic features of malignant lymphomas in Bahraini patients whom diagnosed during the period between January 1996 and December 2001 at the Salmaniya Medical Complex in Bahrain. Seventy-two cases met the inclusion criteria. This included 24 (33.3%) cases of Hodgkin's disease (HD) and 48 (66.7%) cases of Non-Hodgkin lymphomas (NHL). A young age (median 20 years) at presentation with mixed cellularity histology, lack of extra nodal involvement and rare marrow involvement characterized HD. Immunohistochemical staining in 30 cases showed 26 cases (86.7%) of B cell and 4 cases of T cell origin.

Egypt

Gad-El-Mawla et al. (1983) studied a consecutive group of 242 children with Hodgkin's disease attending the National Cancer Institute in Cairo during the years 1975-1980. Males were found to be predominated representing 76.85% of cases. Age distribution was found to be similar to other African countries with an earlier presentation than the US. The most common histopathologic type was found to be the mixed cellularity (60.74% of patients). Late Stages III and IV represented 63.22%, with a high tumor burden. Celiotomy in 154 cases detected more tissue involvement than clinical assessment. Its results coincided with lymphography in 68% of the cases. It showed seven cases with schistosomal hepatic fibrosis. As schistosomal infestation is still prevalent in rural areas of Egypt, celiotomy seems mandatory in the cases studied to accomplish proper staging.

[See also: Jordan > Amr et al., 1986].

Iraq

[See: Jordan > Amr et al., 1986].

Jordan

Amr et al. (1986) malignant neoplasms diagnosed in children through the period of five years (1975-1979).  The most frequent tumors encountered were found to be lymphomas comprising 28.7%, preceded by leukemias (19.6%) and brain tumors (16.1%).  The cohort was divided into three thirds comprising Hodgkin’s disease, Burkitt’s lymphoma, and lymphocytic lymphoma.  Patients with non-Hodgkin’s lymphoma experienced frequent abdominal involvement.  Amr et al. (1986) stated gaining similar results to those described from neighboring countries including Kuwait, Iraq, Saudi Arabia, and Egypt, while contradicting the results obtained from developed countries where leukemias and brain tumors were found to be more frequent than lymphomas.

AlMasri (2004) conducted a study between January 1996 and September 2002 to evaluate the pattern of Hudgkin’s Lymphoma (HL), identify the epidemiological features in Jordan and to compare it with the patterns in developing countries.  A total of 75 patients were included in the study.  The age of the patients ranged from 3-72 years with a median of 20 years.  The male to female ratio was 1.7:1, but among children it was higher (3.75:1).  The largest group was the young adults (15-34 years) accounting for 46% of all cases, the childhood group accounted for 26%, and the adult group accounted for 18%.  The classic HL accounted for 91% of all cases, half of these cases were mixed cellularity (MC) type, and 46% were nodular sclerosis (NS) type. The result showed that MC and NS types of HL accounts for the vast majority among HL North Jordanian patients, similar to the other developing countries.  AlMasri (2004) concluded that HL in Jordan seems to have an intermediate pattern between developing countries and the west.

Kuwait

Motawy and Omar (1986) conducted a study between 1968 and 1981 to represent their experience and philosophy in the management of the Hodgkin's disease in children in Kuwait. A total of 78 children younger than age 15 years were diagnosed, staged, treated, and followed up for a minimum period of two years. Most cases (64%) were found in their first decade and the male-to-female ratio was found to be 1.9:1. Mixed cellularity type was found in 49% and nodular sclerosis in 32%. Lymphocytic depletion type was found to be rare and occurred in only 4% of the cases. More than one half (53%) had found to have Stages III and IV at presentation. The mediastinum was found to be involved in 38%. The treatment of Stages I and II was mainly by radiotherapy, and Stages III and IV by combination of radiotherapy and chemotherapy. The total survival was found to be 75% and relapse-free survival was found to be 53%. Since 1975, lymphography became a routine investigation and staging laparotomy was performed in selected case. The mantle field was extended to include the para-aortic bar and spleen in Stage II with enlarged mediastinum, Stage IIIs after laparotomy, and cases in which laparotomy was thought to be indicated, but was not performed. The new policy resulted in marked improvement in survival (from 56% to 87%) and relapse-free survival (from 32% to 70%). The stage at presentation was found to be the main prognostic factor, although in the second period of the study, the difference between Stage I and II disappeared.

Al-Bahar et al. (1996) studied all cases of Hodgkin's disease reported in the Kuwait Cancer Registry between 1980 and 1989, and compared the age-specific and age standardized incidence rates for the two periods 1980-1985 and 1985-1989. There were a total of 153 and 213 cases of Hodgkin's disease in the two periods, indicating that HD incidence was almost stable in the two periods. The incidence of mixed cellularity subtype was shown to decrease, while the incidence of modular sclerosis increased with time. Al-Bahar et al. (1996) referred these changes to be related to the improvement in the socio-economic conditions.

[See also: Jordan > Amr et al., 1986].

Saudi Arabia

[See: Jordan > Amr et al., 1986].

United Arab Emirates

Bener et al. (2001) studied the rates of consanguinity and family history among families of 117 patients with acute lymphocytic leukemia (ALL) and Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), ranging in age from 2 to 14 years, as well as matched 117 controls. Among the 22 HL cases, there were three consanguineous families (14%). Bener et al. (2001) found out that the consanguinity rate in families of patients with ALL is significantly higher and in those with NHL and HL significantly lower than that in the UAE population.

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