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CASE PRESENTATION
本スライドの対象者
専攻医／専門医
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19歳男性の症例発表と病歴
#1.
Case presentation Yoshiro Hadano
#2.
Case：19 yo man C.C.: fever and headache
#3.
History of present illness-1/2 A 19-year-old man was admitted to a hospital because of fever and rash. Two weeks prior to admission he developed mild headache and low-grade fever and was seen at a local clinic, where rapid test for influenza was negative. His symptoms transiently improved with acetaminophen but 8 days prior to admission, he developed fever to 38.5℃ and a pruritic maculopapular rash over his back that spread to his limbs.
入院時の身体所見と検査
#4.
History of present illness-2/2 Two days later he revisited the clinic where chest radiography was clear and clarythromycin was prescribed for presumed upper respiratory infection. He visited the emergency department (ED) the day before admission because of continued high fever of over 39°C, fatigue, and headache but was sent home with acetaminophen because there was no jolt accentuation of the headache, or neck pain even with extreme neck flexion. He returned to the ED the next day with worsening fatigue and was admitted.
#5.
Pertinent negative body weight loss(-) appetite loss (-) chiii(-) rigor(-) sore throat(-) neck pain(-) chest pain(-) dyspnea(-) cough(-) palpitation（-）Abdominal pain(-) vomiting(-) nausea(-) diarrhea(-) constipation(-) lower back pain(-) arthralgia(-) Pertinent positive fever, headache, general fatigue, rash Review of Systems
#6.
His past medical history was notable only for mumps at the age of 10. acetaminophen, clarithromycin, and an herbal medicine, which he was taking for the past several days. no tobacco or illicit drug use and rarely drank alcohol. denied any sexual contact with anyone. worked in a factory reported occasional contact with silver. lived with his parents; there was no family history of tuberculosis or connective tissue diseases. His father is from Miyazaki, Japan (where HTLV-1 is endemic) and has chronic hepatitis C. denied recent animal exposure or recent travel. His childhood vaccinations were up to date.
身体検査結果と皮疹の評価
#7.
General appearance : sick V.S.:Cons:E4V5M6 BT 39.2℃ , BP 136 / 78 mmHg , HR 76 / min reg. RR 20/min SpO2 98 %(room air) HEENT: conj.: not icteric & not pale throat: not reddish, No sinus tenderness LNs: some palpable in his left neck and left axilla 1cm each, elastic hard, and moderate tenderness Physical Examination-1/3
#8.
Lung: clear wheeze(-) crackles(-) Heart: S1(→) S2(→) S3(-) S4(-) Abd: soft & flat, normal bowel sounds no tenderness , Abd: soft & flat normoactive BS Liver: not palpable , liver knock pain(-) Spleen: not palpable Rectal examination：no masses,no tenderness, occult blood:negative. Physical Examination-2/3
#9.
Back:CVA tenderness -/- 【Extremities】 no pitting edema, and clubbing 【Rash】 (next slide) A diffuse but non-confluent maculopapular rash @chest wall, back,medial aspects of bilateral thighs and around the knees. 【Neurological examination】 unremarkable Physical Examination-3/3
血液検査結果と尿検査
#10.
History of present illness-1/2
#11.
Labo data 1/2 CBC WBC 2100/μl （Neut 72 % st 23 % Seg 49 % Ly 19 % Mo 9% Eo 0%） Hb 13.5 g/dl PLT 136000 /ul MCV 85 fl ESR 26mm/h Chemistry BUN 9.8 mg/dl Cre 1.0 mg/dl Na 135 mEq/l K 3.6 mEq/l Cl 101 mEq/l Glucose 101 mg/dl Alb 4.6mg/dl TP 8.6mg/dl T-Bil 0.5mg/dl AST 42 IU/l ALT 27 IU/l ALP 269 IU/L LDH 463 IU/l
#12.
Labo data 2/2 Coagulation PT-INR 1.24 PT% 69.1% APTT 35.4 Urinalysis pH 6.0 Protein 1+, Glucose (-), Ket (-), Bil (-), Blood (-), WBC (-), OG:1.030 RBC 1-4/HPF, WBC 1-4/HPF No casts, poikilocyte
胸部X線と心電図の結果
#13.
Chest X ray, ECG Chest radiography and electrocardiogram were normal.
#14.
Problem Lists ＃Fever and rash ＃mild headache ＃relative bradycardia ＃Bicytopenia ＃LDH ↑
#15.
What is youR differentials? What do you do?
臨床経過と治療方針
#16.
＃Fever and rash ＃mild headache ＃relative bradycardia ＃Bicytopenia ＃LDH ↑
#17.
Clinical course 1/7 The patient was given intravenous fluids and acetaminophen as needed. Blood cultures, urine culture and cytomegalovirus (CMV) and Epstein-Barr virus (EBV) serologies, HBs-Ag, HCV- Ab, HIV-Ab, anti-muclear antibody (ANA), complements and ferritin levels, quantiferon-TB were ordered. urinary antigen test for legionella was negative. Contrast-enhanced computed tomography (CT) scan of the chest and abdomen was normal except for mild splenomegaly and an enlarged left axillary lymph node.
#18.
Clinical course 2/7 On the third hospital day, WBC count was 1800/μL with 67% neutrophils, 22% lymphocytes, and 1% atypical lymphocytes; LDH rose to 623 IU/L. He had continued fatigue and high fever while the rash gradually fainted with oral anti-histamines and steroid ointment. On hospital day 4, bone marrow biopsy and skin biopsy of his left thigh were performed.
#19.
Clinical course 3/7 Bone marrow biopsy showed normocellular marrow with no abnormal cells and some activated macrophages with hemophagocytic activity. Skin biopsy failed to show any specific pathology.
#20.
Clinical course 4 /7 His left cervical lymph nodes gradually enlarged during the period from day 4 Ultrasound of the neck showed multiple swollen lymph nodes (left side dominant) with largest diameters of 17mm by 9mm by 31mm. Blood and urine cultures returned negative, CMV and EBV serologies revealed previous infection and the ferritin level was 578ng/ml (normal, 39-340ng/ml). Toxoplasma serology and HTLV-1 antibody were ordered.
リンパ節生検の結果と診断
#21.
What is youR differentials? What do you do?
#22.
Clinical course 5 /7 On hospital day 9, an excisional biopsy of his left anterior cervical lymph nodes was performed.
#23.
LN biopsy paracortical foci with necrosis and a histiocytic cellular infiltrate consistent with subacute necrotizing lymphadenitis
#24.
Clinical course 6 /7 On hospital day 9, an excisional biopsy of his left anterior cervical lymph nodes was performed, which revealed paracortical foci with necrosis and a histiocytic cellular infiltrate consistent with subacute necrotizing lymphadenitis (or Kikuchi-Fujimoto disease). Toxoplasma and HTLV-1 antibodies returned negative.
最終診断と経過観察
#25.
Final Diagnosis subacute necrotizing lymphadenitis (Kikuchi-Fujimoto Disease)
#26.
Clinical course 7/7 His condition began to improve after hospital day 9 without specific treatment, including his blood cell count and LDH level. He was discharged home on hospital day 15. The patient was seen in the outpatient clinic 1 and 3 month later and found to be fully active without recurrences of any symptoms or laboratory abnormalities. When seen in follow-up, his WBC count was 6600/μL and LDH was 268 IU/L.
Kikuchi-Fujimoto病の概要
#27.
Kikuchi-Fujimoto disease ・Kikuchi-Fujimoto disease (KFD), also called Kikuchi's disease, is a benign histiocytic necrotizing lymphadenitis described by both Kikuchi and Fujimoto in 1972. ・It seems more common in Asia, especially Japan, where at least 143 cases have been reported since 1972. ・The etiology has not been determined, but a viral cause—including EBV, and human herpesvirus 6 and 8—has been suggested.3 An autoimmune etiology is also implicated because of infrequent association with SLE.
#28.
・The common presentation is low-grade fever with unilateral cervical lymphadenopathy. ・Although generalized lymphadenopathy can occur, it is rare. Other common clinical manifestations include malaise, joint pain, rash, arthritis, and hepatosplenomegaly. ・ No specific laboratory tests for diagnosis are available, but leukopenia (seen in 43% of patients), increased erythrocyte sedimentation rate (40%), and anemia (23%) may be observed. ・ In this case, atypical lymphocytes were seen, and are reported in one-third of patients.
#29.
KFD is generally diagnosed by lymph node biopsy, which typically shows irregular paracortical areas of coagulation necrosis that can distort the nodal architecture, while different types of histiocytes are observed at the margin of necrotic areas. Differential diagnosis - lymphoma, tuberculosis, SLE, and metastatic adenocarcinoma. ・KFD is self-limited; symptoms typically resolve within 1 to 4 months. Patients with severe manifestations have been treated with anti-inflammatory drugs and steroids. A recurrence rate of 3% to 4% has been reported.
臨床的な重要ポイント
#30.