Primary hepatic lymphoma (PHL) is a very rare malignancy and constitutes 0.016&#x0025; of all cases of non-Hodgkin&#x2019;s lymphoma and 0.4&#x0025; of extranodal non-Hodgkin&#x2019;s lymphoma. We describe a rare case of primary hepatic lymphoma presenting as liver abscess which was complicated with the development of a hepatogastric fistula. A 58-year-old man presented with clinical signs of sepsis, high-grade fever, right upper abdominal pain, and weight loss which had progressed in the past 8 months. Noncontrast abdominal computed tomography (CT) revealed a heterogeneously hypodense lesion in the left lobe of the liver with multiple air foci within, which are seen to extend into the body of the stomach. The patient was initially misdiagnosed as a case of rupture of liver abscess into the stomach. Postoperative liver biopsy examination confirmed a diagnosis of diffuse large B-cell lymphoma. Systemic staging revealed no evidence of nodal or bone marrow involvement, so PHL was diagnosed. Chemotherapy was initiated, but discontinued due to the patient&#x2019;s general condition. Finally, the patient succumbed to neutropenic fever following chemotherapy. Here, we present the exceptional case of a primary hepatic lymphoma with an unusual complication, a hepatogastric fistula, and try through the existing literature to show the difficulties involved in diagnosis and treatment.

Mbarek Yaka

Farid Chehab

Mohmed Allaoui

Abdelmonaim Ait Ali

Aziz Zentar

Postsurgical Diagnosis of an Unusual Case of Primary Hepatic Lymphoma Presenting as Liver Abscess with an Uncommon Complication: A Hepatogastric Fistula

Central diabetes insipidus (CDI) is a rare reported complication of acute myeloid leukemia (AML). The onset of AML-associated CDI often precedes the diagnosis of AML by weeks or months and is considered an adverse prognostic indicator in this setting. The mechanism of AML-associated CDI is not known; however, it is often reported in the setting of cytogenetic events resulting in MDS1 and EVI1 complex locus protein (MECOM) gene overexpression. Here, we describe a case of new onset CDI which preceded a diagnosis of AML by 1 month. We detail the clinical and laboratory evaluation of the patient&#x2019;s CDI, and we describe the pathological and laboratory workup of their AML, which ultimately yielded a diagnosis of AML with myelodysplasia-related changes. Additional cytogenetic findings included the identification of the t (2;3)(p23;q27), which leads to MECOM gene overexpression and which to our knowledge has not previously been reported in the setting of AML-associated CDI. The patient received induction chemotherapy followed by allogeneic hematopoietic stem cell transplantation but experienced disease relapse and passed away nine months after initial diagnosis. We emphasize that new onset CDI can occur as a rare complication of AML where it portends a poor prognosis and may be related to AML subtypes displaying MECOM gene dysregulation.

Stephanie L. Pritzl

Daniel R. Matson

Mark B. Juckett

David J. Ciske

Concurrent Central Diabetes Insipidus and Acute Myeloid Leukemia

Bisphosphonates have evolved over the past decades from oral to more potent intravenous preparations. Along with significant paradigm shift in the management of myeloma over the past years, stronger nitrogen-containing bisphosphonates, due to their antiresorptive action on the bones, have found their way as a key and integral part in the management of bone disease in myeloma. Multiple randomized controlled trials have established efficacy of bisphosphonates in reducing skeletal-related events in myeloma. Some well-documented adverse events include acute-phase reactions, esophageal irritation, and osteonecrosis of the jaw. Across all clinical indications, the incidence of inflammatory eye reactions after bisphosphonate infusion ranges from 0.046&#x0025; to 1&#x0025;. However, data from myeloma patients are extrapolated from few reported cases in literature with varying management strategies including discontinuation, switching to different forms, and rechallenging with steroid cover. Inflammatory eye reactions can vary from self-limiting conjunctivitis and episcleritis to serious uveitis and vision-threatening orbital inflammation. We present a similar case of a patient with IgG kappa myeloma who developed flu-like symptoms followed by severe orbital inflammation within 48&#x2013;72&#x2009;hours after receiving zoledronic acid infusion. The patient was successfully managed with intravenous methyl prednisolone followed by oral tapering dose of steroids and discontinuation of further bisphosphonate therapy. A complete recovery was noted in a week&#x2019;s time.

Rehman Faryal

Amjad Hayat

Keeping an Eye on Bisphosphonate Therapy in Myeloma: A Case Report of Ocular Inflammation Postzoledronic Acid Infusion

Anaplastic large cell lymphoma (ALCL) is a rare variety of non-Hodgkin&#x2019;s lymphoma with diverse morphologic variants. Due to the similarity of the different variants with other lymphoma entities, misdiagnosis may be inevitable when immunohistochemical and cytogenetic techniques are not available and histology alone is employed. We report a case of a 43-year-old woman with a seven-month history of neck swelling which was complicated by ulceration of the mass and pathological fracture of the right clavicle after two months delay in arriving at a correct diagnosis. Several attempts to arrive at definitive diagnosis using histology alone gave divergent reports which all misdiagnosed the case until it was sent to a facility outside the country. Our report highlights the limitations and challenges of histology in making a definitive diagnosis of ALCL and the overt importance of immunohistochemical and cytogenetic techniques which are largely unavailable in resource-constrained environment typical of tertiary centers in Nigeria and most sub-Saharan Africa countries.

Oluomachi Charity Nnachi

Innocent Paul Ezenwenyi

Augustine Ejike Okoye

Chinedu Obasi Akpa

Chukwuma Joseph Uzoigwe

Gabriel Chima Ugwu

Diagnostic Challenges of Anaplastic Large Cell Lymphoma in a Resource-Limited Setting: A Case Report and Literature Review

Autoimmune cytopenia, a known paraneoplastic complication of lymphoid neoplasms, may occur before, concurrently, at relapse, or even years after completion of lymphoma treatment. In the case of Hodgkin lymphoma (HL), it is thought that immune dysregulation, typical of this neoplasm, may be involved in the genesis of these manifestations. We report a 57-year-old male presenting with stage IIIA, International Prognostic Score (IPS) 4, nodular sclerosis HL, and severe AA (SAA) confirmed on the histologic exam of the bone marrow that showed severe marrow hypoplasia due to a decrease in the elements of the three cell linages with left shift of the myeloid maturation. Immunosuppression with steroids and cyclosporine A was started. Eltrombopag and G-CSF were also added. In spite of prompt initiation of immunosuppressive therapy, the patient presented an unfavorable outcome with progressive pancytopenia and severe acute cerebral hemorrhagic event. The patient died 59 days after admission. Although autoimmune disorders are described in HL, its concomitant diagnosis is extremely rare. Our case shows a rare instance of SAA as the first manifestation of HL.

Cl&#xe1;udia L. Pedrosa

Patr&#xed;cia Rosinha

Patr&#xed;cia Seabra

Gisela Ferreira

Cl&#xe1;udia Rosado

Lu&#xed;sa Regadas

Cl&#xe1;udia Casais

Jorge Coutinho

Severe Aplastic Anemia as First Manifestation of Classical Hodgkin Lymphoma

Essential thrombocythemia (ET) occurs predominantly in the elderly, but approximately 20% of patients are &lt;40 years old. Unlike other myeloproliferative neoplasms, ET occurs more commonly in women. We encountered a 38-year-old women diagnosed with ET who exhibited elevated platelet count in early pregnancy. Her platelet count exceeded 1500 × 109/L by late pregnancy; interferon <svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.2063904pt" id="M1" height="6.1673pt" version="1.1" viewBox="-0.0498162 -5.96091 7.51131 6.1673" width="7.51131pt"><g transform="matrix(.013,0,0,-0.013,0,0)"><path id="g113-223" d="M545 106L524 126C493 85 467 65 455 65C438 65 427 113 405 238C448 295 498 362 543 439L533 448L478 435C453 386 423 331 398 295H395C370 404 347 448 282 448C169 448 23 309 23 153C23 54 65 -12 128 -12C203 -12 283 70 339 155H341C360 29 380 -12 411 -12C444 -12 491 11 545 106ZM333 204C265 95 210 54 169 54C137 54 113 96 113 171C113 302 191 405 252 405C301 405 318 306 333 204Z"/></g></svg> was administered but failed to induce an adequate response. She underwent emergency cesarean delivery at 37 weeks of gestation. Although her platelet count was 1000 × 109/L immediately after delivery, it markedly increased to 3271 × 109/L approximately 2 weeks later. Cytoreductive therapy was resumed; the subsequent course was free from complications. Several review articles have indicated that because platelet counts of patients may again increase to the pregnancy level or rebound after delivery, cytoreductive therapy should be administered if necessary. However, there is insufficient information on when therapeutic interventions are necessary and how they should be performed. It remains unknown whether the platelet count will decrease after some time without treatment if it rebounds. We hope management guidelines will be established by collecting detailed data on the postpartum course as well as during pregnancy.

Yoshinori Hashimoto

Rina Hosoda

Hiromi Omura

Takayuki Tanaka

Marked Rebound of Platelet Count in the Early Postpartum Period in a Patient with Essential Thrombocythemia

The present study describes a patient aged 70 with very high-risk AML who successfully received a nonmyeloablative matched unrelated donor allograft shortly following SARS-CoV-2 infection, which manifested with mild cough, interstitial abnormalities on chest CT, and pancytopenia with profound bone marrow biopsy histological alterations. In parallel, our study provides bone marrow biopsy data in a series of contemporary patients with serious haematological diseases who had a bone marrow biopsy performed within two weeks of PCR confirmation of SARS-CoV-2 infection. This study is notable because there are no published data describing the bone marrow biopsy changes observed in patients with haematological malignancies and SARS-CoV-2 infection. Finally, it is suggested that nonmyeloablative hematopoietic stem cell transplantation for very high-risk haematological malignancies can be successfully performed following recovery from SARS-CoV-2 infection.

Maria Kaparou

Zbigniew Rudzki

Hannah Giles

Vidhya Murthy

Swathy Srinath

Rebecca Lloyd

Maria Zahid Ahmed

Beena Salhan

Saleena Chauhan

Bhuvan Kishore

Richard Lovell

Claire Horgan

Shankara Paneesha

Evgenia Xenou

Anand Lokare

Joanne Ewing

Hansini Dassanayake

Emmanouil Nikolousis

Alexandros Kanellopoulos

Management of Allogeneic Stem Cell Transplantation for High-Risk AML following SARS-CoV-2 Associated Pancytopenia with Marked Bone Marrow Biopsy Alterations

The portal vein could be occluded by blood clots partially or completely causing portal vein thrombosis (PVT). The acute episode may be asymptomatic or manifested by abdominal pain, increasing body temperature, and unspecific dyspeptic symptoms. The main causes of PVT are categorized into local, acquired, and genetic thrombophilic factors. To our knowledge, this is the 2nd recognized case of PVT &#x2009;following colectomy for colonic inertia successfully treated with an effective anticoagulation therapy. The patient received unfractionated heparin as soon the diagnosis was implemented. The patient was a 34-year-old lady with chief complaint of severe abdominal pain, nausea, vomiting, and anorexia 10 days after the first hospital admission for subtotal colectomy due to colonic inertia. Spiral abdominal CT &#x2009;scan with intravenous (IV) contrast showed thrombosis in main portal vein with its extension to right and left intrahepatic branches. Our case showed that we should keep in mind PVT in patients who present with upper gastrointestinal symptoms several days after a major surgery (open colectomy) as a risk factor and oral contraceptive pills (OCP) usage, postpregnancy, and immobility as other risk factors, that the protein C, S, and FVL deficiencies were secondary, and that the PVT can be managed by low molecular weight heparin plus oral warfarin therapy in the continue.

Mohammad Bagher Jahantab

Saadat Mehrabi

Vahid Salehi

Lotfolah Abedini

Mohammad Javad Yavari Barhaghtalab

Portal Vein Thrombosis following Total Colectomy due to Colonic Inertia: A Case Report and Evaluation of Risk Factors

Introduction. The underlying pathophysiology of haemophagocytic lymphohistiocytosis (HLH) is characterised by excessive inflammation and tissue destruction secondary to abnormal immune activation. The term primary HLH refers to a genetic abnormality that predisposes to the condition whereas secondary refers to HLH being triggered by an underlying condition such as infection (often Epstein Barr Virus), autoimmune, or neoplastic disease. Its variable clinical presentation poses an obstacle to prompt diagnosis in the elderly patient. Case. A 70-year-old Caucasian man was admitted to hospital from a convalescence center with symptoms of fatigue, fever, decreased oral intake, and increasing shortness of breath on exertion. The patient was three weeks after coronary artery bypass grafting. Over the next two weeks, the patient continued to deteriorate both clinically and biochemically. The patient met criteria for haemophagocytic lymphohistiocytosis, likely driven by EBV infection. Bone marrow biopsy supported the diagnosis with evidence of active phagocytosis. The patient was commenced on high-dose dexamethasone and reviewed by haematology with further molecular testing confirming the diagnosis. Discussion. LH is becoming more common in older patients. We propose that new guidelines be developed to aid its prompt diagnosis in this age group.

Eoghan Burke

Patricia Harkins

Jennifer Kieran

Haemophagocytic Lymphohistiocytosis in an Elderly Patient after Recent Cardiac Surgery

Mixed phenotype acute leukemia (MPAL) is a rare hematological malignancy that lacks consensus on optimal management. We report for the first time two cases of treatment-na&#xef;ve B/myeloid MPAL patients treated with a novel chemo-free regimen using venetoclax combined with hypomethylating agents, which successfully induced complete remission with tolerable toxicities.

Di Wu

Wenlan Chen

Zhichao Chen

Qiubai Li

Venetoclax Combined with Hypomethylating Agents for Treatment-Na&#xef;ve B/Myeloid Mixed Phenotype Acute Leukemia

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Case Reports in Hematology publishes case reports and case series in all areas of hematology, including general hematology, pathology, and oncology, with a specific focus on lymphomas and leukemias.

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Case Reports in Hematology