https://wcms.jejunu.ac.kr/medsci/ The Journal of Medicine and Life Science due tolymphoma-relatedsecondarymyelofibrosis. nostic modalityandtreatmentapproachforsevereanemia Received: July15,2020;Revised:28, Accepted: August 6,2020 life-threatening conditions. gic etiologies is ararecondition in the ED, but it can cause marrow failureduetosecondarymyelofibrosisbyoncolo treatments areimportantforpatients’ prognosis.Bone ential diagnosisofthecauseanemiaandappropriate tered intheemergency department patient withangioimmunoblasticperipheralT-cell lymphoma Severe anemiacausedbysecondarymyelofibrosis ina eISSN: 2671-4922 https://doi.org/10.22730/jmls.2020.17.3.103 The andLife JournalofMedicine Science Vol. 17,No. 3,103-106,December 2020 Oncology patients with anemia are frequently encoun Case Report E-mail: [email protected] Tel: 82-647171924,FAX: 82-647171168 Medicine 15, Aran 13-gil,Jeju -si, Jejudo63241,RepublicofKorea Department ofEmergency Medicine,JejuNationalUniversitySchool of Correspondence to :JeongHoKang Key words: marrow Bone failure, Angioimmunoblastic T-cell lymphoma,Anemia, Diagnosis diagnostic approaches andtreatment. of varioushematologic manifestations oftherare andknowledge condition oflymphomaare essential for appropriate After combination chemotherapy, hishematologic profile lymphomaimproved. andunderlying Better understanding sequential combination chemotherapy was introduced despite the presence of severe anemia and thrombocytopenia. lymphoma involvement. myelofibrosis A diagnosis ofsecondary related lymphoma to the underlying was made, and marrow Bone anemia andthrombocytopenia. examination revealed apackedmarrow fibrosis withmarked and lymphoma. Intravenous immunoglobulinwas alsoadministered in combination dueto combined immunehemolytic and,examination was subsequently, immediately performed dexamethasone was initiated to control theunderlying acute severe anemia. findings Thewere laboratory strongly suggestive ofbonemarrow failure syndrome.Bone marrow becauseofanalteredlymphoma hadrecently level department visited ofconsciousness theemergency causedby andcauselife-threatening events.lymphoid malignancy A58-year-old mandiagnosed withangioimmunoblastic T-cell Abstract 2 1 Jaemin Jo Department ofEmergency Medicine,JejuNationalUniversitySchool of Medicine,Jeju,RepublicKorea Department ofInternalmedicine,JejuNationalUniversitySchoolMedicine,Jeju,RepublicKorea 1 , JeongHoKang Bone marrowBone failure, such as aplastic or myelophthisic anemia, can occur due to an underlying INTRODUCTION 1,2) We hereindescribethediag
(ED); hence,differ 2 ,
intake andgeneral weakness since a week ago andvari mental status. The patienthadcomplaintsofpoororal tertiary universityhospitalin anotherprovince. up becausehewantedtoseek forasecondopinionfrom T evaluation andafinaldiagnosisofangioimmunoblastic ized lymphnodeenlargement. Hewasadmittedforfurther and chestcomputedtomography with abdominal discomfort and weight loss, and abdomen Two weeks before his admission,hepresentedto the ED ation inthedrowsylevelof consciousness two days ago. excisional biopsy. However, thepatientwaslosttofollow- -cell lymphoma A 58-year On arrivaltotheED,patient hadthefollowingvital old man was admitted to the ED due to drowsy -old manwasadmittedtotheEDduedrowsy
(AITL) wasmadeafternecklymphnode CASE REPORT Copyright
The Journal of Medicine andLife ofMedicine The Science Journal
(CT) revealedgeneral
Jaemin Jo, Jeong Ho Kang
μL
Figure 1. Changes in hemoglobin and platelet levels according to treatments during hospitalization (Hb, hemoglobin; Plt, platelet; IVIG, intrave nous immunoglobulin; CHOP, combination therapy of cyclophosphamide, doxorubicin, vincristine, and prednisone).
signs: blood pressure, 115/60 mmHg; body temperature, low level of reticulocytes and combined autoimmune hemo- 38.7℃; pulse rate, 92 beats/min; respiratory rate, 20 brea lytic anemia (AIHA) and immune thrombocytopenia (ITP) ths/min; and Glasgow coma scale, 9 points. Physical exa was also considered due to the positive Coombs tests results mination revealed a severely dehydrated state and anemic and low haptoglobin. conjunctiva. Brain CT was performed to differentially diag After bone marrow examination to evaluate etiology, we nose the cause of the altered consciousness, but no specific administered high-dose steroids and intravenous immuno structural lesions were observed. Initial laboratory tests globulin transfusion to the patient, which was marginally The Journal of Medicine and Life Science Journal of Medicine and Life The showed the following results: leukocyte count, 3,300/μL; effective (Fig. 1). After the confirming minimal lymphoma Science Journal of Medicine and Life The hemoglobin (Hb), 2.6 g/dL; platelet count, 67,000/μL; mean involvement and marked secondary myelofibrosis from a corpuscular volume, 87.6 fL; mean corpuscular hemoglo bone marrow examination after a few days of hospitaliza bin, 29.2 pg; red cell distribution width, 13.1%; lactate tion, the combination CHOP (cyclophosphamide, doxo dehydrogenase, 701 IU/L; total bilirubin, 1.3 mg/dL; blood rubicin, vincristine, and prednisone) regimen was very urea nitrogen, 24.6 mg/dL; creatine, 0.9 mg/dL. The results cautiously introduced, and his hematologic profile was of other laboratory tests were within normal limits. dramatically improved. After completing the CHOP che Compared with the Hb level of 9.4 g/dL two weeks ago, motherapy, the patient recovered with nearly normalized acute progression of the severe normocytic anemia was hematologic profiles and was discharged without serious noted. To differentially diagnose the cause of anemia, we complications. performed additional anemia workup and evaluated whether the anemia was due to acute blood loss such as in gastro intestinal bleeding. The anemia workup results showed a DISCUSSION haptoglobin level of below 8 mg/dL and reticulocyte value of 0.06%. Indirect and direct Coombs tests were both posi Anemia is defined as a decrease in blood Hb concentra tive, and the cold agglutinin titer was 1 : 32. Additionally, tion or RBC mass, thereby decreasing the oxygen-carrying peripheral blood smear revealed pancytopenia with rou capacity of the blood. The World Health Organization defi leaux formation without evidence of leukoerythroblastic nes anemia as a Hb level of less than 13 g/dL in adult males reaction. Fe, folate, and vitamin B12 levels were within and 12 g/dL in adult females, which includes the definition normal limits, and there was no evidence of acute blood of anemia as the lowest 2.5% of Hb levels in a healthy pop loss. As a result of the ED evaluation, bone marrow failure ulation.2) Anemia is a common condition that is diagnosed such as aplastic anemia related to underlying lymphoid based on laboratory test results in patients visiting the ED. malignancy was initially suspected based on the extremely In most cases, anemia is a sign of an underlying disease and
104 https://wcms.jejunu.ac.kr/medsci/ https://wcms.jejunu.ac.kr/medsci/ Uncommon hematologic manifestation of lymphoma
is not the final diagnosis. Therefore, the goal of diagnostic treatments effectively improve cytopenia caused by bone evaluation in the ED is to determine the underlying cause marrow fibrosis.10-12) Therefore, we gradually introduced of the anemia. Moreover, emergency physicians should combination chemotherapy with a reduced dose and obser carefully evaluate life-threatening conditions that require ved a remarkably improved hematologic profile. Subse timely treatment.2-4) In case of anemia with other hemato quent bone marrow biopsy also revealed normal bone mar logic abnormalities such as other cytopenia or abnormal row findings after several cycles of CHOP chemotherapy. cells on peripheral blood smear, an unusual cause of the disease should be suspected. Pancytopenia or bicytopenia is caused by various types of etiologies including congenital CONCLUSIONS bone marrow failure syndrome, idiopathic aplastic anemia, nutritional deficiency, sequestration, autoimmune disease, In this case report, we presented a diagnostic approach and bone marrow infiltrative disease. Therefore, the differ and treatment for severe anemia caused by rare etiologies ential diagnosis should include these disease entities, and in patients with lymphoma. In many patients with cancer, disease-specific diagnostic approaches are required.5) anemia is frequently confirmed by routine laboratory tests. In this case, AITL was diagnosed by histological eval However, rare but fatal hematologic complications caused uation and immunophenotyping of the lymph node upon by lymphoma are difficult to diagnose without physician’s biopsy. AITL is characterized by monoclonal proliferation suspected diagnosis. Better understanding of the rare clini of the follicular T helper cell expressing CD3 and CD4. cal manifestations of lymphoma is essential for appropriate AITL represents only 1%~2% of all non-Hodgkin lym diagnostic approaches and effective treatment for patients phoma cases. AITL occurs mainly in elderly people in their with life-threatening hematologic complications. 60s and 70s, and the most common presenting symptom is generalized lymphadenopathy, which is often accompanied by B symptoms (fever, weight loss, and night sweats).6) ETHICAL APPROVAL Currently, anthracycline-based chemotherapy is considered the first-line therapy for AITL. There was no difference in This study was approved by the institutional review overall survival rate when comparing different anthra board (IRB) of Jeju National University Hospital (IRB No. The Journal of Medicine and Life Science Journal of Medicine and Life The cycline-based regimens: CHOP, ACVBP (dose-intense 2020-05-009). The requirement for informed consent was doxorubicin, cyclophosphamide, vindesine, bleomycin, waived by the IRB. and prednisone), or mBACOD (methotrexate, bleomycin, doxorubicin, cyclophosphamide, vincristine, and dexa Conflict of Interest: No potential conflict of interest rele methasone). In AITL patients treated with first-line therapy, vant to this article was reported. complete remission rates were reported as 61%, with five- Funding/Support: None declared. year overall survival and recurrence-free survival rates rep orted as 32~33% and 18%, respectively. High-dose chemo- therapy followed by autologous stem cell transplantation REFERENCES can improve the likelihood of complete remission.6,7) AITL can be disguised as an immune activator with a 1. Swenson KK, Rose MA, Ritz L, Murray CL, Adlis SA. Recogni high erythrocyte sedimentation rate and lead to positive tion and evaluation of oncology-related symptoms in the emergency autoimmune tests such as rheumatoid factor, antinuclear department. Ann Emerg Med 1995;26:12-7. factor, and anti-smooth muscle factor. Thus, AITL can 2. Vieth JT, Lane DR. Anemia. Emerg Med Clin North Am 2014;32: cause various hematologic abnormalities associated with 613-28. autoimmune reactions such as warm-type AIHA, ITP, and 3. Blackman SC, del Rey JAG. Hematologic emergencies: acute ane 8,9) lymphocytopenia. In this case, the main cause of hemato- mia. Clin Pediatr Emerg Med 2005;6:124-37. logic abnormalities including severe anemia and thrombo 4. Long B, Koyfman A. Emergency medicine evaluation and manage cytopenia was bone marrow failure caused by lymphoma- ment of anemia. Emerg Med Clin North Am 2018;36:609-30. related secondary myelofibrosis, with AIHA and ITP con 5. Weinzierl EP, Arber DA. The differential diagnosis and bone marrow tributing in part. This etiology of cytopenia is rarely mani evaluation of new-onset pancytopenia. Am J Clin Pathol 2013;139: fested by lymphoma patients and difficult to suspect ini 9-29. tially.5) Previous studies have reported that anti-lymphoma 6. Lunning MA, Vose JM. Angioimmunoblastic T-cell lymphoma: the
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