Two cases with hepatic amyloidosis suspected of having primary sclerosing cholangitis

Sclerogenic biliary changes in hepatic amyloidosis are seldom observed. Here, we report two recent cases initially suspected as primary sclerosing cholangitis (PSC), which were later diagnosed as hepatic amyloidosis (AL type). Case 1: On the basis of magnetic resonance cholangiopancreatography (MRCP) findings, PSC was suspected in a 41‐year‐old woman with jaundice. Computed tomography (CT) showed nodular pulmonary lesions and swollen cervical, mediastinal and para‐aortic lymph nodes, the cause of which was unknown despite detailed examinations. Because of rapid deterioration in the patient's liver function, living donor liver transplantation was performed. She was then diagnosed with hepatic amyloidosis, but died of heart failure due to cardiac amyloidosis 74 days after surgery. Case 2: On the basis of MRCP findings, PSC was suspected in a 49‐year‐old woman with jaundice. CT showed multiple cystic pulmonary lesions, and hypogammaglobulinemia was also observed (immunoglobulin G, 481 mg/dL). After a biliary plastic stent was placed, liver and lung biopsy confirmed the presence of amyloid deposition. These two cases indicate that it is important to consider hepatic amyloidosis as a differential diagnosis of PSC. The presence of atypical extrahepatic lesions may be useful clues for confirming the diagnosis.     

Histiocytic necrotizing lymphadenitis or Kikuchi's disease. CT radiologic features

Histiocytic necrotizing lymphadenitis, Kikuchi Fujimoto's disease (KFD) is characterised by fever and lymphadenopathy, usually large cervical, unilateral lymph nodes. Such clinical presentation demands a work-up to exclude serious medical conditions like malignancy and infections. Foci of necrosis with lymphocytic Histiocytic predominance in association with scarce polymorphonuclear cells on lymph node examination, confirm the diagnosis of KFD. Many different patterns of computed tomographic (CT) appearance of KFD have been reported. We describe the CT scan finding in two patients with this disease. All our cases showed, after two and three weeks of evolution respectively, enlarged lymph nodes with hypodense centres and peripheral ring enhancement. These radiological alterations correlated with the central lymph node necrosis found in the anatomopathological studies. In conclusion, KFD should be considered in patients with fever, cervical lymph node enlargement and CT scan showing hypointense centres and peripheral ring enhancement.     

EB virus-associated peripheral T cell lymphoma presenting with hemophagocytic syndrome and hepatic cell necrosis

A 72-year-old woman was admitted with left cervical lymphadenopathy, high fever, pancytopenia and liver dysfunction. Bone marrow aspiration showed infiltration of large atypical lymphoid cells and hemophagocytic histiocytes, thus suggesting a diagnosis of lymphoma associated hemophagocytic syndrome (LAHS). An abdominal CT scan revealed multiple low-density areas in the liver, and the patient's liver function rapidly deteriorated. Histologically, the cervical biopsy showed lymphoma cell infiltration with prominent necrosis and karyorrhectic debris. The lymphoma cells expressed CD3+, CD4-, CD8+, CD20-, CD56+/-, TIA-1+, granzyme B+, and EBER was positive using in situ hybridization. DNA analysis of the TCR beta and gamma chain gene with the Southern blot showed rearranged bands. These findings were compatible with those of EB-virus associated peripheral T-cell lymphoma. After chemotherapy with the THP-COP regimen, the patient's liver dysfunction improved rapidly, but she died from bacterial peritonitis due to perforation of a recurrent duodenal ulcer. Post-mortem examination of the liver showed multiple irregular massive necroses of the hepatocytes, where no lymphoma cell infiltration was present. Hemophagocytic histiocytosis was remarkable in the bone marrow, spleen, lymph nodes, and liver. Marked elevation of serum levels of cytokines such as TNF-alpha or IFN-gamma suggests that these cytokines played an important role in the pathogenesis of the hepatic cell necrosis.     

Plasma exchange and continuous hemodiafiltration as an initial treatment for diffuse large B-cell lymphoma-associated hemophagocytic syndrome

A 68-year-old man was admitted to our hospital because of fever, jaundice and hepatosplenomegaly. A diagnosis of diffuse large cell, B-cell type malignant lymphoma, associated with hemophagocytic syndrome (LAHS), was made. CT scan revealed lymphadenopathy in the abdominal cavity and multiple tumors in the spleen. Performance status and hepatic coma grade were 4 and II, respectively. Laboratory findings showed bicytopenia (Hb 9.9 g/dl, platelet 35 x 10(3)/microliter), severe liver dysfunction (ALP 1,115 U/l, gamma-GTP 437 U/l, T.Bil 15.4 mg/dl, D.Bil 12.8 mg/dl) and elevated levels of beta 2 microglobulin (12.9 mg/dl), ferritin (2,300 ng/ml) and sIL-2 receptor (36,900 U/ml). Plasma exchange (PE) and continuous hemodiafiltration (CHDF) enabled the patient to undergo diagnostic procedures, irradiation (total 34 Gy) and chemotherapy. Biopsy specimens revealed infiltration of lymphoma cells into the liver and bone marrow. We measured the blood concentrations of TNF-alpha, IL-6, and IL-8 before and after PE and CHDF by the ELISA method, and found normalization of hypercytokinemia after the procedure. It was suggested that initial treatment with PE and CHDF was effective for control of HPS, enabling us to perform chemotherapy for the lymphoma.     

组织细胞坏死性淋巴结炎~(18)F-FDG PET/CT表现:2例报告及文献复习

目的提高对组织细胞坏死性淋巴结炎(histiocytic necrotizing lymphadenitis HNL)18F-FDG正电子发射断层显像/计算机断层成像(positron emission tomography/computed tomography,PET/CT)的认识。方法报道本院2例病理确诊的组织细胞坏死性淋巴结炎的PET/CT表现,并检索复习文献。结果 2例患者都有高热,大部分检查结果无明显异常,其中1例颈部淋巴结明显肿大,抗感染治疗无效,怀疑淋巴瘤,PET/CT检查发现患者大部分受累淋巴结18F-FDG最大标准摄取值(SUVmax)2.50,摄取18F-FDG最高的部位在颈部,SUVmax最高达8.45。颈部淋巴结活检,病理证实为HNL,激素治疗有效。复习文献,发现摄取18F-FDG最高部位的淋巴结活检阳性率高。结论组织细胞坏死性淋巴结炎患者在PET/CT上多表现为淋巴结摄取18F-FDG异常增高,颈部淋巴结受累最多见,不易与淋巴瘤相鉴别。但PET/CT可以发现全身淋巴结受累情况,尤其对于深部淋巴结或体积较小的淋巴结,有助于活检定位。     

Case of multiple intrapulmonary lymph nodes]

A 45-year-old man who had hypertension, hyperthyroidism, and bronchial asthma was admitted to our hospital because of a low-grade fever and chest pain. The physical findings and laboratory data were almost all within normal limits except for evidence of mild inflammation and liver dysfunction. The chest X-ray findings seemed normal, but a computed tomography (CT) scan showed multiple nodules in both lower lung fields. We suspected the cryptococcosis or lung cancer. Biopsy by video-assisted thoracoscopic surgery (VATS) yielded a diagnosis of multiple intrapulmonary lymph nodes. In cases with the above radiologic findings, careful attention should be paid to making the differential diagnosis between intrapulmonary lymph nodes and primary lung cancer. The promotion of diagnostic imaging and advances in techniques have made it easier to identify small peripheral nodules in the lungs, and we now know of their existence. Solitary intrapulmonary lymph nodes are encountered frequently, but multiple or increasing numbers of nodes, as in our case, are very rare. Moreover, because cases with elevated CEA levels have been reported, differentiation from lung cancer appears to be important.     

Microscopic polyangiitis initiated with liver dysfunction, calf pain and fever of unknown origin

We report herein a case of microscopic polyangiitis (MPA), presenting onset with a spiking fever, liver/biliary dysfunction without jaundice and calf pain without elevation of serum creatine phosphokinase. During 1 month of careful examinations for initial diagnosis, the patient developed renal dysfunction and pulmonary hemorrhage. Based on the results of positive MPO-ANCA, renal and pulmonary involvements, the patient was diagnosed with MPA and treated with high-dose prednisolone and oral cyclophosphamide. Soon after initiation of the treatment, symptoms such as fever, calf pain, liver/biliary dysfunction and renal dysfunction disappeared with decrease of MPO-ANCA titer to the normal level.     

La cholécystite xanthogranulomateuse, un diagnostic histologique inattendu: à propos d’un cas

Xanthogranulomatous cholecystitis (XC) is an uncommon form of chronic inflammation of the gallbladder characterized by a thickening of the gallbladder wall. It may clinically and radiographically mimic gallbladder carcinoma. In spite the progress of imagery, preoperative diagnosis is still often difficult which can lead to inappropriate extended surgery. We herein report the clinical presentation of a 71-year-old man with no medical history who was admitted to our department with a chief complaint of intermittent pain in his right upper abdomen, radiating to the back since a year. There was no history of jaundice, fever, anorexia, or weight loss. Physical examination revealed no signs of cholecystitis. Routine laboratory tests including white cell counts were within normal ranges. For further examination, we performed an abdominal ultrasonography. It revealed a thickening of the gallbladder wall on the hepatic side. There was no associated biliary dilatation or gallstones. CT scan of the abdomen showed similar findings with regional enlarged lymph nodes. The boundary of the gallbladder and liver parenchyma was ill defined. The patient was discharged in good condition on the 7^th day following surgery. He is now in good health eight months after surgery. As imaging findings were strongly suggestive of malignancy, we decided to perform a radical surgery. Peroperative finding shows a gallbladder mass invading the liver bed with some regional lymph nodes. We performed subsegmentectomy of the liver S4a + S5 and lymph node dissection. Histological examination showed diffuse chronic inflammatory cell infiltration with focal lipid-laden macrophage accumulation in the gallbladder wall and conclude on chronic xanthogranulomatous cholecystitis with no evidence of malignancy. The patient was discharged in good condition on the 7th day following surgery. He is now in good health 8 months after surgery. Xanthogranulomatous cholecystitis is a rare destructive inflammatory disease of the gallbladder which may be indistinguishable from a malignant neoplasm on the basis of imaging findings. The clinical presentation is similar to that of cholecystitis. A correct preoperative diagnosis of XC is important to avoid unnecessary laparotomy.     

原位肝移植术后肝脓肿的诊断和治疗

目的 探讨原位肝移植术(OLT)后肝脓肿的病因、诊断、治疗和预防措施。方法自1993年1月至2003年6月，本中心共行OLT274例，术后并发肝脓肿6例(2．2％)。患者主要临床表现有发热、寒战、腹痛、身目黄染、肝功能损害、低蛋白血症、贫血、白细胞及中性粒细胞比例增高等。诊断主要根据临床表现及超声或CT检查。治疗方法主要包括脓肿抽吸引流、清除胆泥．抗炎和支持治疗。结果6例中2例治愈，3例放弃再次肝移植术，2例死于严重全身感染，治愈率为33．3％。结论OLT后肝脓肿病因较复杂，可能与肝动脉血栓或狭窄、甲基强的松龙冲击治疗、胆管炎症和介入治疗等有关。OLT后肝脓肿的预后较差．应加强预防措施防止其发生。     

A case of infectious mononucleosis complicated with severe jaundice

A 28-year-old male was admitted to our hospital with tonsillitis and jaundice. Laboratory findings showed leukocytosis (rate of atypical lymphocytes was 40%), liver dysfunction and hyperbilirubinemia. Epstein-Barr virus (EBV) viral capsid antigen (VCA) IgM and IgG antibodies were positive, and EB nuclear antigen (EBNA) antibody was negative. Abdominal ultrasonography demonstrated hepato-splenomegaly and swelling of intraperitoneal lymph nodes. A diagnosis of infectious mononucleosis was made due to EBV infection. Conservative therapy was given. Total bilirubin and alkaline phosphatase increased to maximum levels of 10.2 mg/dl and 1,590U/l. A liver biopsy specimen revealed infiltration of lymphocytes in sinusoids and portal areas, focal necrosis and intrahepatic cholestasis in parenchyma. Liver function tests returned to normal limits and EBV VCA IgM antibody became negative within 10 weeks from onset.     

Polycystic liver disease with obstructive jaundice: treatment with ultrasound-guided cyst aspiration.

A 59-year-old woman with polycystic liver (PCLD) and kidney disease (PCKD) presented with jaundice, fever, and abdominal pain. A computered tomographic (CT) scan performed immediately after an endoscopic retrograde cholangiopancreatogram (ERCP) clearly demonstrated a hepatic cyst obstructing the biliary system. The cyst was then aspirated under ultrasound guidance with complete relief of obstruction.     

Hydatid Cyst of the Liver Rupturing into the Biliary Tract—Mimicking Acute Cholecystitis on Hepatobiliary Scanning

Two patients presenting with right upper abdominal colicky pain, jaundice, and fever had a hepatobiliary scan using Tc 99m HIDA. The scan was suggestive of acute cholecystitis and a space occupying lesion in the liver. On operation, liver echinococcosis, located in the right lobe rupturing into the biliary ductal system, was found. Intraoperative cholangiography revealed filling defects in the main biliary ducts. Exploration of the common bile duct disclosed daughter cysts and cystic debris. Acute cholecystitis or stones were not found. We conclude that in patients with a clinical picture and HIDA scanning compatible with acute cholecystitis and a space occupying lesion in the liver, the diagnosis of hydatid cyst of the liver which has ruptured into the biliary tract should be considered.     

Hodgkin's disease diagnosed by endoscopic ultrasound-guided fine needle aspiration of a periduodenal lymph node

Hodgkin's disease rarely presents as obstructive jaundice. We report a case of Hodgkin's disease arising in periduodenal lymph nodes, presenting with biliary obstruction, definitively diagnosed on cytologic material obtained by endosonographically-guided real-time fine needle aspiration biopsy and confirmed at laparotomy. The medical literature pertaining to the use of endosonography and fine needle aspiration biopsy for pancreatic lesions and abdominal lymphoma is reviewed. Currently available data support the use of fine needle aspiration biopsy in establishing the diagnosis of lymphoma. This case highlights the utility of endoscopic ultrasonography with endosonographically guided real-time fine needle aspiration biopsy in diagnosing and managing patients with extrahepatic biliary obstruction or suspected abdominal lymphoma. Pairing endosonographically guided real-time fine needle aspiration biopsy with on-site cytologic assessment and immediate specimen triage can lead to definitive diagnosis of abdominal lymphoma, avoiding surgical intervention in many cases.     

Giant cell hepatitis: an unusual cause of fulminant liver failure

Giant cell hepatitis is a very rare disease of unknown origin. It has been hypothesized that drugs, viral infections, or autoimmune reactions may play a pathogenetic role. Here, we describe a 33 year old patient with bacterial bronchitis who was treated with doxycycline (100 mg/d) for one week. Furthermore the patient complained of malaise and a distinct jaundice. Liver parameters increased dramatically (AST 4670 U/l, ALT 5350 U/l, bilirubin 226 μmol/l) and liver function was impaired (INR = 1,45). The ultrasound scan showed a hepatomegaly with no signs of cirrhosis, normal spleen size and normal bile ducts; liver perfusion was normal. No evidence of Wilson's disease, hemochromatosis, autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis, hepatitis A, B, C and E, HIV, CMV, VZV, adenoviral infections, or paracetamol intoxication was found. Subsequently, the patient developed acute liver failure (AST 2134 U/l, ALT 2820 U/l, bilirubin 380 μmol/l, INR 3.0) and a beginning renal failure. Therefore, he was transferred to our transplant center. Due to increasing confusion and somnolence due to cerebral edema mechanical ventilation was needed. Because of an acute renal failure and severe hepatic encephalopathia MARS-hemodialysis was performed. Three weeks after the appearance of the jaundice he underwent liver transplantation (MELD 40). Surprisingly, in the explanted liver the diagnosis of giant cell hepatitis was made. Today--2 years after successful liver transplantation--the patient is in very good condition with normal liver function. In conclusion, giant cell hepatitis is a rare cause of acute liver failure that is often recognized only histologically.     

Necrotizing lymphadenitis presenting as mesenteric lymphadenopathy]

A 15-year-old girl was admitted to our hospital because of fever, diarrhea, and right lower abdominal pain on November 11, 1997. Computed tomographic and ultrasound studies of the abdomen disclosed enlarged mesenteric lymph nodes. Hematologic and serologic findings included WBC 3000/microliter, LDH 550 IU/l, IFN-gamma 264 pg/ml, IL-6 9.74 pg/ml, sIL-2R 781 U/ml, and ferritin 720 ng/ml. Although the patient was treated with antibiotics, high fever and abdominal pain persisted with progressive anemia and leucocytopenia (1800/microliter). Bone marrow aspiration specimens revealed an increase of histiocytes with phagocytosis. Appendectomy and lymphadenectomy were performed on November 21. A lymph node specimen showed necrosis with infiltration by large mononuclear cells. The resected appendix revealed reactive lymphoid hyperplasia. Based on these findings, a diagnosis of necrotizing lymphadenitis (NL) was made. The postoperative course was satisfactory and systemic symptoms resolved gradually without specific treatment. However, high fever and abdominal pain recurred with right cervical lymph node swelling on December 15. The patient's general condition improved after treatment with prednisolone. In NL, lymphadenopathy is usually observed in the cervical region, and the involvement of intra-abdominal lymph nodes is quite rare. Our findings indicated the possibility that IFN-gamma may play an important role in the pathogenesis of NL with hemophagocytic syndrome.     

Obstructive jaundice associated Burkitt's lymphoma mimicking pancreatic carcinoma

The primary compromise of the pancreas in lymphomas is uncommon. However, in advanced stages of Non-Hodgkin's lymphomas (LNH) the secondary invasion of the pancreas is observed more frequently. Jaundice due to extrahepatic cholestasis as a presentation form is extremely rare, with only few cases described in the literature. The aim is to present a case of an obstructive jaundice as an expression of Burkitt's lymphoma probably due to a diffuse pancreatic infiltration in an adult without immunodeficiency with a rapid response of cholestasis to low dose of hydrocortisone. Skin tumor simultaneously present with jaundice allowed the histologic diagnosis with skin biopsies. After a unique dose of 100 mg hydrocortisone, jaundice improved and cholestatic enzymes decreased, pancreas became smaller and common bile duct diameter became normal at ultrasound and CT scan, also skin tumors turn pale and diminished in size. There are isolated reports of Burkitt's lymphoma cases with associated obstructive jaundice due to pancreatic infiltration or by compression by lymph nodes of the bile ducts, many of them are pediatric cases or immunodepressed HIV patients. In the case presented, surgical resection of the pancreatic infiltration and biliary drainage, either surgical or endoscopic during the same procedure was not necessary for the histopathologic diagnosis of the illness like is described in the literature. The diagnosis was suspected by the rapid decrease of cholestatic features after a single dose of hydrocortisone and the histology was easy done by a skin biopsy. We think the interest in this case is the quick response to low doses of corticoids, which avoided the necessity of surgical procedure for the diagnosis of the biliary tree obstruction, allowing a quick implementation of the specific chemotherapeutic treatment of the lymphoma without any surgical or endoscopic procedures to heal the jaundice.     

Kikuchi's necrotizing lymphadenitis: a cause of fever of unknown origin and splenomegaly

Kikuchi's disease is characterized by lymphadenopathy in young patients and may be mistaken for malignant disease both clinically and histologically. We report the case of a 26-year-old man with persistent fever for 3 weeks and splenomegaly, in whom pancytopenia developed afterwards. A bone marrow aspiration was normal. Blood, urine, throat, stool and bone marrow cultures were negative as were serological tests for lues, Toxoplasma, Epstein-Barr virus and Widal's test. An abdominal CT scan showed enlarged retroperitoneal lymph nodes and an exploratory laparotomy was performed. Two lymph nodes were excised and a wedge biopsy of the liver was performed. The histological findings in the lymph nodes were compatible with the diagnosis of Kikuchi's disease. The patient became afebrile on the 2nd postoperative day without any treatment. He has been well for 4 months after discharge.     

Clinical vignette in antiretroviral therapy: jaundice

HIV caregivers face many challenges following initiation of ART. The development of jaundice is uncommon but worrisome. In this case, two distinct and contrasting episodes of jaundice were observed. In the first instance, isolated elevation of the indirect bilirubin without elevation of the alkaline phosphatase was noted. The normal PT and serum aminotransferase levels indicate the absence of intrinsic liver dysfunction. Elevations in the indirect bilirubin may result from either impaired uptake/conjugation or excess production. The latter, usually from acquired hemolysis, may be a complication of an occult NHL. A work-up for this AIDS-related malignancy was not initiated since the caregivers recognized jaundice as a complication of IDV, which inhibits UDP-glucuronyl transferase and produces a Gilbert's-like syndrome. Physicians can expect to encounter this syndrome even more frequently with ATV. Experienced patients given RTV-boosted ATV have experienced elevations of unconjugated hyper-bilirubinemia in up to 45 percent of cases in clinical trials. However, such elevations do not reflect liver dysfunction and symptomatic jaundice requiring dosage reduction that occurred infrequently (7 to 8 percent of study patients). Counseling patients about this syndrome may promote adherence and prevent self-directed interruptions of ATV that compromise efficacy. The second case of jaundice provides a more formidable diagnostic challenge. The triad of LFT abnormalities (mild elevation of aminotransferases, normal PT, and marked cholestatic jaundice) implies an acute process that is mildly toxic to hepatocytes without affecting their synthetic function. The subacute nature of the patient's cholestatic jaundice suggests either intrahepatic infiltrative disease of the liver or extrahepatic obstruction of the biliary tree, most likely due to the patient's relatively modest level of pain and lack of fever. Despite LFT abnormalities occurring 17 months after a switch in his ART, cumulative drug-related toxicities must still be considered. Ritonavir can produce significant elevations in the AST/ALT, especially with pre-existing chronic liver disease as with hepatitis C virus coinfection. The NRTIs can produce hepatic steatosis, a result of mitochondrial toxicity and impaired fatty acid oxidation. However, jaundice and cholestasis are not typical of the latter syndrome. With a negative contrast CT that excludes parenchymal liver disease, investigation of the biliary tree to assess the presence of AIDS-related cholangitis was the next step. Performing a sphincterotomy or stent placement, and obtaining brushings or biopsy specimens to determine the extent of extrahepatic obstruction may help define a pathogen and be life-saving. The negative results of the ERCP justify the final diagnostic step, a liver biopsy to evaluate microscopic infiltrative disease that might not have been detected on contrast abdominal CT. Examples might include granulomatous disease (MAC), fungal etiologies (histoplasmosis), carcinomatosis (lymphoma, hepatoma, cholangiocarcinoma), and microvascular disease (bacillary angiomatosis). The failure to observe granulomatous inflammation in the liver does not exclude MAC infection, as MAC may involve other peri-aortic or mesenteric lymph nodes. This form of IRIS is unlikely given the abdominal CT findings, lack of systemic complaints, and extended persistence of liver aminotransferases. The nonspecific results of the liver biopsy are a common outcome in advanced AIDS patients with elevated alkaline phosphatase levels. Despite not having identified a pathogen, the biopsy establishes chronic liver disease and prompts re-evaluation and change of treatment to NFV. The subsequent normalization of the patient's aminotransferase levels suggests a prior adverse effect of LPV/r in the setting of unexplained, chronic liver disease. Most importantly, this case highlights the importance of HIV caregivers to review ART for safety when noting chronic liver dysfunction. Patients need to be counseled to minimize acetaminophen use, to consume alcohol in moderation, and to avoid behavior with risk for hepatitis C. Finally, all HIV patients should receive appropriate vaccination against hepatitis A and B if serology shows lack of protective immunity.     

Biliary tuberculosis mimicking cholangiocarcinoma: treatment with metallic biliary endoprothesis

A 58-yr-old patient who presented with obstructive jaundice was evaluated with ultrasonography (US), computed tomography (CT), and percutaneous transhepatic cholangiography (PTC). Diffuse irregular stenosis of the extrahepatic bile ducts and periductal ill-defined soft tissue density along the hepatoduodenal ligament was determined. The patient was originally misdiagnosed with cholangiocarcinoma and, because the extent of disease process made surgical bypass impossible, was treated with a percutaneously inserted metallic stent. Histopathological examination of the endoluminal biopsy revealed ductal tuberculosis (TB). Most of the previous reports in the literature indicated that biliary obstruction was due to enlarged tuberculous lymph nodes compressing the bile duct. To our knowledge, only three cases of biliary stricture due to tuberculous involvement of the bile ducts were reported previously. This case illustrates the importance of tissue diagnosis in all cases of obstructive jaundice to avoid missing rare but curable diseases.     

Hodgkin's disease diagnosed from the numerous Reed-Sternberg cells in the bone marrow

A 46-year-old female was admitted to our hospital for fever and weight loss in September, 1986. Physical examinations were unremarkable. CBC revealed moderate anemia and leukopenia with abnormal lymphocytes. Examination of the bone marrow (BM) disclosed peroxidase negative blasts and multinucleated or multilobulated giant cells positive for CD30 (Ki-1) antigen. Chest X-ray was negative. CT scan and echography of the abdomen showed minimal enlargement of retroperitoneal lymph nodes (LN). Lymphangiography revealed mild enlargement of the LN without filling defects. Gallium scan was negative. Hence a diagnosis of Hodgkin's disease (HD) stage IVB was made. She was treated with MOPP therapy with modification and obtained a complete remission. BM involvement of HD occurs mostly in advanced stages. We assumed that this is a rare case of HD in which bone marrow metastasis occurred in a very early stage of the disease or that of bone marrow primary.