Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto or Kikuchi necrotizing lymphadenitis, is a rare self-limiting noncancerous infection of the lymph nodes. This disease was first reported by Kikuchi and Fujimoto independently in 1972 in Japan. It results in protruded lymph nodes around the neck, accompanied by night sweat and mild fever. Histiocytic necrotizing lymphadenitis commonly and clinically manifests itself as cervical lymphadenopathy. An autoimmune disorder, whereby the body immune system attacks and destructs healthy body tissues, is one of the suggested causes of Kikuchi disease. Infectious causes have also been proposed as another cause of this disease. These suggestions hold even though no known sure causes of Kikuchi have been discovered yet. This condition often heals on its own treated or untreated, within a period of four months. Initially, reports of Kikuchi-Fujimoto were confined to Japan, until 1982 when other cases of the condition came from Germany. Up to date, only four cases have been reported the Kingdom of Saudi Arabia.
Retrospective Case review
Case 1: An analysis of the five-year period of histological diagnosis of Kikuchi disease in Riyadh Central Hospital in Saudi Arabia revealed that there were 3 Saudis and ten non-Saudis diagnosed with the disease. In the determination exercise, the female to male ratio was 1.16:1 (7 women to 6 men), involving persons with a mean average age of 27.5 (14 to 36) years. Cervical lymphadenopathy was the most common clinical symptom in the four cases with 77% dominance; fever was the second feature with 61.5%, and Leukopenia decreases in leukocytes, occurred in 53.7% of patients. Only one patient showed bilateral enlargement of lymph nodes, a condition known as bilateral hilar lymphadenopathy. Cases of fibrin thrombi in the capillaries reported 76.9% ((Abba, Afzal, Al-Moharab & Baez-Giangreco, 1995)). These are the clinical and laboratory data for 13 cases; Cervical unilateral (9), cervical bilateral (1), axillary bilateral (1), thorax hilar bilateral (1) and generalized conditions (1).
The histologic observation on the distribution of lymph nodes in the 13 cases identified areas of necrosis without granulocytes. The lymph node pattern in the patients was preserved to a limited extent. The cortex, paracortex and the medullary cavities were the focus areas of necrosis without a particular preference to any of the three. Further, observation showed the presence of karyorrhexis with dispersed deposits of fibrin in the necrotic region. Vascular blockage with well-structured thrombi was evident in two cases while vasculitis with lymphocytic infiltration was observed in one instance. Ten (58.8%) of such situations showed the presence of germinal centers while in 76.9% of such cases, the presence of fibrin thrombi was noted (Abba, et al. 1995).
Another review of patient follow-up information of the same cases showed that nine patients recovered from the lymphadenopathy and related symptoms without treatment. Follow-up information on the remaining four patients was not found.
Case 2: Next reviewed histopathological report on all lymph nodes in the period between 1990 and 2003 in the Kingdom of Saudi Arabia provides additional information on the prevalence of Kikuchi-Fujimoto Disease (KFD). Fifteen cases out of 2500 biopsies conducted, were diagnoses of KFD. In concurrence with other cases reviewed previously (Abba, et al. 19995), the majority were females, since the female to male ratio was 2.7:1. The participants were between the ages of 13 and 46, an average of 29 years (Al-Maghrabi & Kanaan, 2005). Axillary lymphadenopathy condition was present in one patient while the rest were diagnosed with cervical lymphadenopathy. Within a period of up to 10 years of patient follow-up, no sign of recurrent KFD was noted. The tests for the Bcl-2 protein that regulates cell death or apoptosis, and tumor suppressor protein, p53 were negative. The test for Ki-67 protein, a cellular marker for cell proliferation or cell division, came out positive in 11 of the 15 cases reviewed. The results of these tests show that cells that regulate apoptosis are not significant in the detection of KFD, unlike the cell proliferation antigen Ki-67.
Case 3: A similar retrospective study of all patients, especially adults, admitted to Riyadh Medical Complex, Riyadh, Kingdom of Saudi Arabia between April 1996 and March 2000 confirmed what other medical researchers in the developed countries have found out involving lymphadenopathy (Abba, Bamgboye, Afzal & Rahmatullah, 2002). The research investigated 258 cases, where 145 (56.2%) were Saudi nationals, and the rest were from other countries. The mean average age was 35.2 years. The females, 153 (59.3%) in number, were the majority while the males, 105 (40.7%) were the minorities. The symptoms of local swelling were the majority in 195 (75.6%) cases. Fever as a symptom was noted in 85 (32.9%) cases. Fever was also associated with other symptoms like sweat, pain and weight loss. There was no clear-cut difference between malignant and benign disorders, confirming the fact that lymphadenopathy is easy to confuse with other ailments having similar symptoms such as malignant lymphoma and tuberculosis. Recurring in this study, and most importantly, is the fact that Kikuchi disease affects more young females than males and affects mostly the cervical group of lymph nodes. Once again, it is prudent to conclude that KFD shares clinical features with systemic lupus erythematosus (SLE), such as fever, leukopenia, and arthralgias (Abba et al., 2002).
Castleman's Disease: Background
Castleman's disease, also known as Castleman disease (CD), giant lymph node hyperplasia or angiofollicular lymph node hyperplasia (AFH) is a rare lymph node disease first described by Benjamin Castleman in the 1950s. Though not cancer, CD manifests itself from abnormal overgrowth of cells in the lymph tissues just like lymph node cancer. Castleman disease is categorized in different ways depending on the extent to which it affects the human body. Unicentric or localized Castleman disease affects one type of lymph nodes causing abnormal growth usually in the chest and abdomen regions where particular lymph nodes occur. The growth can progress on to the trachea causing swellings around the neck areas leading to difficulties in breathing or eating. Such extensions are eliminated through surgery. Multicentric Castleman disease (MCD) is the second type, and it attacks various groups of lymph nodes as well as the organs bearing lymph node tissues. It is also common with people suffering from HIV/AIDS. Symptoms range from fever, nerve damage, and loss of weight, weakness, and night sweats. Other categories depend on how CD looks when viewed through a microscope lens, such as microscopic subtypes of CD. The treatment of CD, in addition to surgery, is often done through radiotherapy or chemotherapy.
Retrospective Case Reviews
Due to its rarity, not very many cases of Castleman's disease have been diagnosed in Saudi Arabia Unlike other areas of the world. One of the few cases that have been reviewed in Riyadh Hospital and Research Center, Saudi Arabia, presented a 70-year old female patient of MCD with progressive nodal disease related to autoimmune thrombocytopenia. Attempts to treat the ailment using steroid medication were unsuccessful until an intravenous immunoglobulin which was administered to her brought a momentary success. Her platelet count improved gradually after that with weekly doses of the anti-CD-20 antibody also known as rituximab. Administration of the antibody provided an alternative treatment measure for Castleman disease especially when it is immune related (Ibrahim K., 2011).
While unicentric and multicentric are the most common types of Castleman's disease clinically, four cases of eye, orbital or ophthalmic involvement have been reported in some literature review. Less than 15 cases of orbital CD have been reported whereby some have been confirmed by biopsy and others have not. The confirmed case reported orbital CD in a 12-year old girl having single lesion and proptosis that had remained dormant after two decades of diagnosis (Guerero et al., 2016). Medical researchers from King Khaled Eye Specialist Hospital and King Saud University Medical City, Riyadh, in collaboration with other international researchers, present two cases of orbital lymph node hyperplasia.
Case 1: In 2008, a 38 year old female complained of a progressive left eye proptosis that had started seven earlier. Images from a Computed tomography scan of the problem showed an orbital lump in the inferonasal area. After undergoing an incisional biopsy, lymphoid follicles with hyperplastic germinal center came to view via the histopathological division. Mature plasma cells, large follicles, and atypical vascular stroma were observed in the follicular area. The conclusion was that plasma cell variant (PCV) of Castleman disease had affected the orbit and the patient was referred for chemotherapy in 2015 (Galindo-Ferreiro & Galvez-Ruiz A, et al. 2016).
Case 2: This case introduced a woman of 40 years of age, in 2007, who had complained of left eye proptosis and swollen eyelid for a whole year. After ophthalmic tests had been carried out, the results showed enlarged lacrimal gland. Additional orbitotomy and excisional biopsy were done on her left side revealed abnormal lymphoid follicles with atrophic germinal centers. The final results confirmed hyaline vascular variant (HVV) of Castleman's disease (Galindo-Ferreiro et al., 2016). To further understand the problem, more tests were conducted on the patient that exhibited the involvement of chest, abdomen, and neck lymph nodes indicating the presence of multicentric Castleman's Disease. The following year, she was referred for systemic medical care.
It is rare for CD to affect the orbital area, unlike the mediastinum, as these two cases found out. Various other cases were scrutinized by the investigators to see if lymphoma was involved. In the 40 cases reviewed for an ocular or periocular association, conjunctiva membrane took 42.5%, the orbital portion was 25%, and lacrimal gland 12.5% of the cases. Importantly, only 3 cases, an equivalent of 7.5%, were categorized as Castleman's disease. Alkatan, et al. (2013) observes that orbital CD has a propensity to affect males within an age bracket of 17 and 70 years and above. On the contrary, the reported cases, in this case, cite females of about 40 years of age. Castleman's disease can be repressed with early diagnosis and intervention through methods like surgical excision, radiation therapy, and adjunctive chemotherapy.
Kimura diseases: Background
Kimura disease (KD) is a chronic, rare, benign inflammatory disorder with an angiolymphatic proliferation of a mysterious etiology. This disease mainly presents itself as a pain-free lymphadenopathy or subcutaneous tissue in the neck regions or head regions. Kimura disease was first reported in 1937 in Japan by Kimm and Szeto who called it "eosinophilic hyperplastic lymphogranuloma." It was not until 1948 when it gained its current status and name when Kimura, Yoshimura, and Ishikawa took notice of its vascular component and how it exhibited "unusual granulation combined with hyperplastic changes in lymphoid tissues." Even though it is so common in Asian males and can trigger neoplasm, Kimura disease affects population with an irregular pattern, randomly. Often confused with Angiolymphoid hyperplasia with eosinophilia (ALHE) - another rare vasoproliferative condition, medical researchers, and authors believe Kimura disease is a serious version of ALHE. The distinctive features of the two conditions depend on the histopathologic...
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