A case of elderly-onset systemic lupus erythematosus (SLE) complicated with severe liver dysfunction and pancytopenia due to myelofibrosis

A 67-year-old woman was admitted to our hospital with a fever. She had been experiencing arthralgia for about one month. On admission, she had a fever of 38.5 degrees C, was anemic and was experiencing tenderness in the joints of both hands, elbows and feet. Laboratory data revealed proteinuria, urinary cylinders, pancytopenia (WBC 900/mm3, Hb 9.5 g/dl, Plt 7.8 x 10(4)/mm3), liver dysfunction (GOT 414 IU/l, GPT 140 IU/l), and hyper-gamma globulinemia. Antibiotics and granulocyte-colony stimulating factor were administered intravenously. Bone marrow aspiration was unsuccessful, but a bone marrow biopsy revealed bone marrow fibrosis. Immunological examinations were positive for antinuclear antibodies, anti-deoxyribonucleic acid (DNA) antibodies, anti-double stranded anti- DNA antibodies, as well as a decreased level of serum complement and an increased level of serum immune complexes. Tests for viral antigens and antibodies known to cause hepatitis were negative. Based on these findings, a diagnosis of SLE accompanied by liver dysfunction and bone marrow fibrosis was made. Steroid pulse therapy was initiated, but her liver function deteriorated on the first day of steroid therapy, and she died three days later. SLE accompanied by myelofibrosis is extremely rare, and only 17 and cases have been reported to date. Among these reports, the present case is the second oldest subject and the first SLE patient to suffer from both myelofibrosis and severe liver dysfunction.     

A case of Plummer disease that appeared in older old age after 10-year course of subclinical hyperthyroidism

A 81-year-old woman with a thyroid tumor and subclinical hyperthyroidism since ten years ago was admitted to our hospital for palpitations and hyperthyroidism (FT(4) 1.75 ng/dl, FT(3) 5.37 pg/ml, TSH<0.03 microIU/ml). Although thyroid stimulating antibody (TSAb) was transiently and mildly positive, anti-TSH receptor antibody (TRAb), microsome test, and thyroid test were negative. Thyroid echogram showed an isoechoic nodule in the left lobe (33 x 42 x 22 mm) and a small nodule (10 x 15 x 9 mm) in right lobe. Thyroid scintiscan showed a hyperfunctional (hot) nodule in left thyroid lobe with suppressed uptake in the remainder of the gland. The uptake rate of thyroidal radioiodine ((123)I) in 24 hours was within the normal range (7.3%). Based on the above findings, a diagnosis of Plummer disease was made. Since she refused invasive surgical or radioiodine treatment, she was treated with 10 mg thiamazole daily. After treatment with propranolol and thiamazole, the thyrotoxic symptoms disappeared and thyroid function returned to normal level. She had osteoporosis but she had neither atrial fibrillation nor cardiac symptoms. This was a rare case of Plummer disease that appeared in extremely old age after a long course of subclinical hyperthyroidism.     

Dietary supplement implicated in fulminant hepatic failure in a well-controlled Wilson disease patient

We encountered a patient with previously well-controlled Wilson disease who experienced fulminant hepatic failure with hemolytic anemia, possibly caused by the dietary supplement Health Proportion^® (Jubilant Co., Ltd., Ehime, Japan). A 21-year-old woman was admitted to our hospital with marked liver dysfunction and severe hemolytic anemia. Free serum copper level was elevated at 101 μg/dl, and urinary copper excretion was extremely increased (25,600 μg/day). Plasma exchange and continuous hemodiafiltration were performed to remove serum copper and to treat the hemolytic anemia. However, liver function did not improve, and she underwent liver transplantation on 28th day after admission. Copper and iron contents in the resected liver were high at 851.9 μg and 551.7 μg/dry liver weight (g), respectively, despite the patient having regularly taken d-penicillamine since diagnosis and having a well-controlled copper level 1 year before her admission. Two months before admission, the patient had taken a dietary supplement made from soybeans for 1 month. This supplement was labeled as containing large amounts of copper and iron, and we assume that this caused fulminant hepatic failure with hemolytic crisis in this patient. It is important to be mindful of the micronutrient content of dietary supplements, especially for metabolic disorder patients.     

Severe hypertriglyceridemia induced by tamoxifen

A 71-year-old woman was admitted to our hospital because of severe hypertriglyceridemia. The patient had a 26-year history of non-insulin-dependent diabetes mellitus and hyperlipidemia (T-chol 300 mg/dl, TG 300 mg/dl). She was treated with sulfonylurea and clofibrate. Seven years before admission, she had undergone a radical mastectomy for cancer of the left breast. After the operation, she had received tamoxifen and fluorouracil. One month before admission, she had marked hypertriglyceridemia (triglyceride 2,106 mg/dl). After discontinuation of tamoxifen and fluorouracil, her serum triglyceride level decreased to 372 mg/dl; when tamoxifen was given again, it increased to 581 mg/dl, and her hepatic triglyceride lipase activity decreased from 0.228 to 0.164 mumol FFA/ml/min. Apolipoprotein E phenotype was wild type E3/3. The concentration of sex-hormone-binding globulin increased from 110 to 130 nmol/l. These changes associated with tamoxifen treatment were similar to those seen after administration of estrogen. Tamoxifen, an anti-estrogen, has been used as adjuvant therapy in cases of estrogen-receptor-positive breast cancer. Tamoxifen has some weak estrogenic activity. The tamoxifen-induced hypertriglyceridemia seen in this case was an effect of its estrogenic action.     

A case of chronic myelogenous leukemia complicated with nephrotic syndrome

An 81-year-old woman was admitted, complained general malaise, and edema on face and lower extremities. In the peripheral blood, leucocytosis (17,220/mm3), microcytic hypochromic anemia (RBC 348 x 10(4)/mm3, Hb 9.6 g/dl, Ht 29.2%), and thrombocytosis (130 x 10(4)/mm3) were present, and many myeloid cells containing of myeloblasts, promyelocytes and so on were observed. Bone marrow aspiration revealed increment of the myeloid series without hiatus leukemia . The Neutrophil Alkaline Phosphatase score and rate was low, and on bone marrow scintigram using indium chloride, liver and extremities were shown. On admission, proteinuria (21.5 g/dl) and hypoalbuminemia (2.5 g/day) were pointed out, and the renal biopsy specimen showed membraneous proliferative glomerulonephritis (MPGN), so we diagnosed this case that chronic myelogenous leukemia (CML) complicated with nephrotic syndrome. At first, she was treated with prednisolone, but proteinuria was not entirely improved, then busulfan was given, myeloid cells in peripheral blood were disappeared and proteinuria was gradually decreased. From this coarse, the causality between CML and nephrotic syndrome was verified.     

Erythroleukemia in pregnancy with successful outcome of delivery]

A 37-year-old woman was admitted to our hospital because of anemia in the 33rd week of pregnancy. On admission, the hemoglobin was 7.6 g/dl, platelets 508,000/microliters and WBC 8,300/microliters with 4% blast cells. Bone marrow aspirate demonstrated 50% erythroblasts of nucleated cells, which had prominent megaloid and polynucleic changes, and 20% myeloblast cells. We diagnosed her as having erythroleukemia. Receiving packed-red-cell transfusions, she had a cesarean section, and gave birth to a female infant (BW 2,175 g) in the 34th week of pregnancy. At the same time, she had a total hysterectomy with left adnexectomy. The postoperative course was favorable. She had a combination chemotherapy of BHAC-DMP, resulting in a partial remission. After the second induction chemotherapy with subcutaneous use of G-CSF and BHAC-DMP, complete remission was obtained, which lasted for almost 17 months. The child is growing well without any hematological disorder.     

A case of eosinophilic pneumonia possibly due to ifenprodil]

A 69-year-old woman with a history of subarachnoid hemorrhage was started on ifenprodil for dizziness. Three weeks later, fever, cough, chills, dyspnea and skin eruption developed. A chest radiograph showed bilateral ground-glass shadows. Blood tests showed a white cell count of 14,400/mm3 with 32% eosinophils and a C reactive protein (CRP) level of 20 mg/dl. The arterial blood gases on room air were as follows: pH 7.45, PaCO2 33 torr, and PaO2 56 torr (Table 1). Ifenprodil was withdrawn and intravenous meropenem and minocycline administration was started on admission. Her fever improved rapidly and the CRP decreased, but hypoxemia and hypereosinophilia persisted. On the third hospital day, she underwent bronchoscopy with bronchoalveolar lavage (BAL). The differential count of BAL cells was 63% eosinophils, 15% lymphocytes, 21% macrophages, and 1% neutrophils. Intravenous methylprednisolone 250 mg/day for 3 days was commenced, leading to a clinical improvement. She received oral prednisolone (30 mg/day) for the next 4 days, and was then discharged without any symptoms. She has had no recurrence since. Both the drug lymphocyte stimulation test and the skin test for ifenprodil were negative.     

Marked reactive plasmacytosis accompanied by drug eruption in a patient with aplastic anemia

A 61-year-old woman with aplastic anemia was admitted to our hospital in October 2009 because of fever and abdominal pain. She had been treated with cyclosporin A without showing any effect. On admission, uterine cancer was diagnosed and the left uterine appendages were swollen. She was treated with cefepime for febrile neutropenia without effect, and left-sided adnexitis was diagnosed. After cefepime was changed to meropenem, marked plasmacytosis was observed in the peripheral blood (23%) and bone marrow (79%) with the appearance of skin eruption. Although the plasma cells were morphologically abnormal, the cytoplasmic immunoglobulin light chain deviation was not detected by flow cytometric analysis, and M protein was not found by serum immunoelectrophoresis. She was diagnosed with reactive plasmacytosis and treated with dexamethasone. The drug eruption and plasmacytosis improved soon after starting the treatment. Although reactive plasmacytosis is observed with a variety of conditions, including infection, neoplasms, autoimmune disorders, and hemolytic anemia, it has not been reported to accompany drug eruption. Reactive plasmacytosis is sometimes not possible to distinguish from plasma cell neoplasms on morphology alone and needs to be diagnosed comprehensively by using flow cytometric analysis and immunohistochemical evaluation.     

A case of systemic lupus erythematosus with postpartum hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome and concomitant high phosphatidylserine-dependent anti-prothrombin antibody levels

In August 1994, a 19-year-old woman presented to her dermatologist with a slight fever, arthralgia, and a butterfly rash. Discoid lupus erythematosus was suspected, and serological testing yielded positive results for antinuclear antibody. She was diagnosed with systemic lupus erythematosus without organ failure and was treated with only nonsteroidal antiinflammatory drugs. She became pregnant in June 2001, at age 26. In November her obstetrician noted that she had severe hypertension, edema of the low limbs, and proteinuria. On admission, she was diagnosed with severe preeclampsia, and cesarean section was performed. On hospital day 3 the patient developed sudden epigastric pain and vomiting. Laboratory tests revealed thrombocytopenia, liver dysfunction, and microangiopathic hemolytic anemia, leading to a diagnosis of HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Plasma exchange was performed for 5 days. The thrombocytopenia, liver dysfunction, and proteinuria diminished quickly. Later testing revealed a high titer of plasma phosphatidylserine-dependent anti-prothrombin antibody. This case is useful for exploring the relations between SLE, HELLP syndrome, and anti-prothrombin antibody.     

A case of systemic lupus erythematosus with postpartum hemolysis,elevated liver enzymes,and low platelet count (HELLP) syndrome and concomitant high phosphatidylserine-dependent anti-prothrombin antibody levels

Abstract

In August 1994, a 19-year-old woman presented to her dermatologist with a slight fever, arthralgia, and a butterfly rash. Discoid lupus erythematosus was suspected, and serological testing yielded positive results for antinuclear antibody. She was diagnosed with systemic lupus erythematosus without organ failure and was treated with only nonsteroidal antiinflammatory drugs. She became pregnant in June 2001, at age 26. In November her obstetrician noted that she had severe hypertension, edema of the low limbs, and proteinuria. On admission, she was diagnosed with severe preeclampsia, and cesarean section was performed. On hospital day 3 the patient developed sudden epigastric pain and vomiting. Laboratory tests revealed thrombocytopenia, liver dysfunction, and microangiopathic hemolytic anemia, leading to a diagnosis of HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Plasma exchange was performed for 5 days. The thrombocytopenia, liver dysfunction, and proteinuria diminished quickly. Later testing revealed a high titer of plasma phosphatidylserine-dependent anti-prothrombin antibody. This case is useful for exploring the relations between SLE, HELLP syndrome, and anti-prothrombin antibody.     

Successful treatment of autoimmune hemolytic anemia associated with multicentric Castleman disease by anti-interleukin-6 receptor antibody (tocilizumab) therapy

We describe herein the successful treatment of severe autoimmune hemolytic anemia (AIHA) in a patient with multicentric Castleman disease (MCD) by humanized anti-interleukin-6 (IL-6) receptor antibody (tocilizumab) therapy. Inflammatory anemia is commonly reported; however, AIHA is a very rare complication of MCD. In 1996, a 45-year-old Japanese woman was referred to our hospital because of generalized lymphadenopathy, anemia and skin eruptions. Lymph node biopsy demonstrated MCD. She was treated with prednisolone (1 mg/kg/day), which improved the anemia and skin eruptions. In 2009, she suddenly developed Coombs-positive hemolytic anemia. The blood count was as follows: hemoglobin 4.7 g/dl, platelets 490 × 10(9)/l and white blood cell count 9.8 × 10(9)/l. Both direct and indirect Coombs' tests were strongly positive. She was treated with 8 mg/kg tocilizumab every 2 weeks. One month later, her hemoglobin levels rose dramatically to 10.9 g/dl and her haptoglobin level, hypergammaglobulinemia and clinical symptoms had also markedly improved. To the best of our knowledge, this is the first report of the efficacy of tocilizumab in AIHA associated with MCD. The well-established role of IL-6 in the pathogenesis of MCD may have been responsible for the improvement in the AIHA associated with MCD. Anti-IL-6 receptor antibody treatment could be an attractive therapeutic approach for AIHA associated with MCD.     

Intermittent administration of natural interferon-alpha for over 5 years induced complete suppression of Philadelphia chromosome in a patient with myelogenous leukemia]

A 60-year-old woman was admitted to our hospital because of gastric ulcer, anemia, and leukocytosis in November 1984. Blood cell counts on admission were as follows: RBC 407 x 10(4)/microliters, Hb 9.8 g/dl, WBC 33,000/microliters (baso 8%, eo 7%, myelo 11%, meta 2%, stab 4%, seg 54%), Plt 93.7 x 10(4)/microliters. Bone marrow showed hypercellular and myeloid hyperplasia. She was diagnosed as Ph1-chromosome positive chronic myelogenous leukemia. She received natural interferon-alpha at the dosage of 600 x 10(4) IU daily for 22 days from January 14, 1985. After March 1985, she has been given intermittent administration of interferon once in 10 to 20 days, and maintained normal blood cell counts. Cytogenetic improvement was seen on 35 months after the start of IFN and complete suppression of Ph1 chromosome was observed at July 1990 (66 months after).     

Encephalitis in a renal transplantation patient

The patient was a 55 year-old-woman with chronic renal failure due to idiopathic mesngial deposition of Ig A. She received a second allograft of a kidney from a cadaver. Results of a preoperative serologic Ig G tests for EBV and CMV were positive. She was given triple-drug immunosuppressive therapy, consisting of cyclosporine,azathioprine, and steroids. Seven years later, azathioprine was changed to mycophenolate mofetil. One year later, she was admitted to the hospital with a three to four week history of vertigo (which did not improve after sulpiride was administrated) and an influenza-like syndrome. A CT scan of the brain appeared normal, so paroxysmal positional vertigo was the diagnosis. Two weeks after admission to the hospital, the patient reported visual hallucinations and impairment of consciousness. Results of laboratory tests were leukocyte increase (polymorphonuclear leukocytes), anemia, hyponatremia and renal failure. Chest radiography, brain CT, and electroencephalography revealed no pathologic signs. The CSF examination revealed 300 cells/ml (79% PMNL), glucose 63 mg/dl, protein 45 mg/dl. Six hours later the treatment was initiated with ampicillin, ceftriaxone and ganciclovir iv, she experienced seizures that affected the left side of her body, but without interictal recovery. The patient required intubation and mechanical ventilation in the intensive care unit. An MRI of the brain images, revealed high signal-intensity regions indicating lesions on the bulb, protuberance, mesencephalon, left thalamus and parenchyma adjacent to the corpus callosum (fig. 1). Six days later, the patient partially recovered consciousness, and she had not neurologic sequelae. Intubation was terminated. As soon as PCR revealed EBV DNA in CSF samples, the treatment with ceftriaxone and ampicillin was discontinued. Treatment with ganciclovir was maintained for 8 weeks (4 weeks with iv and another 4 weeks with oral treatment). On day 35, the examination of a specimen of CSF revealed: glucose 46, protein 78, 15 cells/ml (100% lymphocytes). The patient went home on day 55 after admission to our hospital. She regained her normal neurologic function. Three weeks later MRI, showed reduction of the size of the lesions and the lesions on the brain stem had disappeared.     

Exacerbation of cranial nerurological symptoms by platelet transfusion before the diagnosis of thrombotic thrombocytopenic purpura

A 47-year-old woman was transported to our hospital because of vomiting and syncope after breakfast. Physical examination revealed icterus and anemia of bulbar conjunctivas, and abnormal neurological findings were detected. Laboratory data indicated marked anemia and thrombocytopenia (Hb 5.2 g/dl, Plt. 0.6×10(4)/μl), but no leukocyte abnormalities were found. Transaminase was slightly elevated, and serum indirect bilirubin in was also elevated. Based on these data, we initially suspected Evan's syndrome, which involves idiopathic thrombocytopenic purpura with autoimmune hemolytic anemia. So we transfused red blood cells, performed platelet transfusion, and administered steroids, but there was no response to these therapies. On the 4th day of admission, she developed a stroke followed by coma. After the stroke, we diagnosed the case as thrombotic thrombocytopenic purpura (TTP) because laboratory findings showed diminished activity of ADAMTS 13 (a disintegrin-like metalloproteinase with thrombospondin type 1 motifs 13) and ADAMTS 13 antigen. It is important to suspect TTP when hemolytic anemia with thrombocytopenia is observed, and to check the activity and antigen of ADAMTS13 immediately for the diagnosis. Platelet transfusion should be done cautiously in these cases.     

Documentation of dynamic electrocardiographic changes shortly after the onset of tako-tsubo cardiomyopathy

A 76-year-old female with atrial fibrillation and prior cerebral infarction had chest discomfort during rehabilitation for left hemiparesis, and visited the nearby hospital. Her ECG, which was obtained 10 min after the onset, showed marked ST-segment elevation in leads I, II, III, aV(F) and V(2-6), and she was referred to our hospital for cardiac examination. On admission, her ECG, which was obtained 50 min after the onset, showed poor R wave progression and mild ST-segment elevation in leads V(5-6). During only 10 min after the admission, the ST-segment level increased dynamically, and it decreased spontaneously again. Left ventriculography showed akinesia of the mid-to-distal portion and hyperkinesia of the basal portion of the left ventricular chamber, and coronary angiography showed no significant coronary artery disease despite of significant ST-segment elevation. We diagnosed her as having tako-tsubo cardiomyopathy. She was discharged well 10 days later.     

Cases from the Osler Medical Service at Johns Hopkins University

A 47-year-old white woman with a history of stage III squamous cell carcinoma of the anus was transferred to Johns Hopkins Hospital for further evaluation of renal failure, hemolytic anemia, and thrombocytopenia. The patient was first diagnosed with squamous cell carcinoma of the anus 1 year before admission. She was treated with external beam radiation of the pelvis and two cycles of mitomycin C-based chemotherapy (a cumulative dose, 34 mg/m(2)). Her clinical course was complicated by Clostridium difficile colitis and myositis successfully treated with prednisone. Three months before admission, the patient developed dysuria. Her creatinine increased from normal to 1.7 mg/dL, and microscopic hematuria was present. A renal ultrasound and an abdominal computed tomographic scan showed no abnormalities or obstruction. One month before admission, she underwent a cystoscopy, which showed only radiation-induced changes in the bladder. Two weeks before admission, the patient became delirious and was taken to a hospital, where she was found to be anemic, with a hematocrit level of 23.7%, and thrombocytopenic with a platelet count of 110,000/mm(3). Her creatinine level was 5.9 mg/dL. Previous values of hematocrit, platelet count, and serum creatinine were normal. On admission at Johns Hopkins Hospital the patient had no complaints. She was afebrile on physical examination and had normal vital signs. Head, neck, chest, cardiovascular, and abdominal examinations were normal. There was skin pallor, but no echymoses or petechiae. She was alert and oriented with normal mental status. Her neurologic examination was normal. Laboratory data showed a white blood cell count of 6390/mm(3), a hematocrit level of 26.5%, and a platelet count of 26,000/mm(3). Her blood urea nitrogen level was 57 mg/dL, creatinine level was 4.0 mg/dL, and lactate dehydrogenase was 550 U/L (reference, 115 to 275 U/L). Urinalysis showed innumerable red blood cells and large protein. A peripheral blood smear showed fragmented red blood cells, schistocytes, no abnormal white blood cells, and few platelets. There was no radiographic or clinical evidence of relapse of her squamous cell carcinoma. What is the diagnosis?     

Progressive hemolytic anemia due to delayed recognition of a Beall mitral valve prosthesis

In addition to a working knowledge of general complications such as thromboembolism and infective endocarditis, optimal care of the patient with a prosthetic valve requires specific knowledge concerning the characteristics of a given patient's prosthesis. This may need to include the ability to identify the valve roentgenographically when history and records are unavailable. A 53-year-old woman with mitral stenosis secondary to rheumatic heart disease and status post a reported Bjork-Shiley mitral valve (MV) replacement 17 years prior to hospital admission was referred for evaluation of severe hemolytic anemia. Previous cinefluoroscopy in 1986 at the time of a cerebrovascular accident revealed a normally functioning caged disc prosthesis and not the tilting disc of a Bjork-Shiley prosthetic valve. The valve was not further characterized and she continued receiving warfarin therapy until May 1989 when she presented with laboratory findings showing a marked hemolytic anemia with a hemoglobin of 6.5 mg/dl and lactate dehydrogenase (LDH) value of 2100 IU. Echocardiography revealed normal valvular function without evidence of perivalvular leak. The patient was referred for further evaluation with chest roentgenogram at the time of hospital admission revealing a valve configuration characteristic of the Beall model 103/104 series that has been found to manifest progressive disc variance with a high degree of hemolytic anemia (despite normal noninvasive evaluation of MV function), disc tilting with intermittent regurgitation, and catastrophic disc embolization in extreme cases. The precise identification of valvular prosthesis in patients after valve replacement is crucial for optimal management. As in our case, the mere identification of a particular valve may necessitate certain management and therapy based on the natural history of that valve. In the absence of reliable history and/or records, the roentgenographic examination should lead to the precise identification.     

Isolated Cerebral Vasculitis in the Unilateral Middle Cerebral Artery in a Case with SLE

A 47-year-old woman, who was diagnosed to have systemic lupus erythematosus (SLE), was admitted because she suffered a severe ischemic stroke three weeks after experiencing a transient attack of aphasia. Diffusion-weighted MRI revealed high intensity at the borderzone of the middle cerebral artery (MCA), while the proximal portion of the left MCA was occluded with its vascular wall enhanced by gadolinium. Intravenous methylprednisolone and heparin were administrated without any symptomatic benefit. She developed severe right hemiparesis with aphasia. Isolated cerebral vasculitis in the large vessel has been rarely reported in SLE patients. The presence of an enhanced vascular wall in the MRI with gadolinium could support the diagnosis.     

Development of severe aplastic anemia in a girl with Hashimoto's thyroiditis and papillary thyroid carcinoma

A 14-year-old girl was admitted because of general fatigue and cervical lymphadenopathy. She showed bilateral struma (IInd degree) and enlargement of her left cervical lymph nodes. Laboratory data revealed neutropenia (219/microliter) and thrombocytopenia (Plt 5.1 x 10(4)/microliter) with mild anemia (Hb 11.1 g/dl), and the bone marrow aspirate and biopsy specimens showed hypocellularity. In addition, auto-antibodies against thyroid peroxidase (TPO) and thyroglobulin (TG) were highly elevated. Computed tomography of the neck showed a nodule in the left thyroid lobe with marked lymphadenopathy, and fine needle aspiration biopsy demonstrated papillary thyroid carcinoma with Hashimoto's thyroiditis and metastasis to the lymph nodes. One month after left thyroid lobectomy and cervical lymphadenectomy, the patient's condition progressed to very severe aplastic anemia, and she received immunosuppressive therapy consisting of cyclosporin A and anti-thymocyte globulin. Hematologically, partial and complete responses were obtained three and six months later, respectively. Of interest, anti-TPO and TG antibody titers remarkably decreased after immunosuppressive therapy. The patient had HLA-DR 2(DRB 1*1501) and DR 8(DRB 1*0802). The former is frequently found in patients with cyclosporin A-dependent aplastic anemia, and the latter is frequently found in Asian patients with Hashimoto's thyroiditis, suggesting an underlying autoimmune background for the simultaneous outbreak of aplastic anemia and Hashimoto's thyroiditis complicated by thyroid carcinoma.     

Phenacetin-induced hemolytic anemia misdiagnosed as unstable hemoglobinopathy]

A 30-year-old woman was admitted in August 1984 with anemia. She had an initial hemolytic attack in 1977. Unstable hemoglobinopathy was suspected. Despite splenectomy in 1979, hemolytic attacks continued. On admission, she was anemic and cyanotic. Hb heat denaturation test was positive. However, the first structure of amino acid of hemoglobin was normal. A large amount of white powder was found in her belongings, and was later identified as phenacetin. N-acetyl-P-aminophenol, a metabolite of phenacetin was demonstrated in her urine. Hemolytic attacks disappeared completely after she stopped taking phenacetin.