Skip to main content
Erschienen in: Forensic Science, Medicine and Pathology 4/2021

20.08.2021 | Case Report

Sudden death due to leukostasis in a subject with undiagnosed chronic lymphocytic leukemia

verfasst von: Lorenzo Gitto, Rohin Mehta, Kanish Mirchia, Christopher Ramos, Michel Tawil, Serenella Serinelli

Erschienen in: Forensic Science, Medicine and Pathology | Ausgabe 4/2021

Einloggen, um Zugang zu erhalten

Abstract

Sudden death due to leukostasis and lymphocyte thrombi in patients with chronic hematologic malignancies is rare. Leukostasis is characterized by highly elevated leukemic cell count and decreased tissue perfusion symptoms, leading to severe complications and even death. Chronic lymphocytic leukemia (CLL) is a chronic lymphoproliferative disorder that shows a highly heterogeneous clinical course, ranging from indolent form to very aggressive disease. Due to its low metabolic and mitotic rate, there is a lower incidence of clinically significant leukostasis in patients with CLL. Two main theories have been proposed in the development of leukostasis: (1) increased blood viscosity due to large leukemic cell populations; (2) high metabolic activity and cytokine production by leukemic cells. Both mechanisms lead to local hypoxic damage.
We present a case of a 70-year-old man who died suddenly in the absence of symptoms. Autopsy and histology examinations revealed findings consistent with CLL and diffuse leukostasis involving the major organs’ vessels.
In the presence of gross and/or microscopic findings suggesting a potential hematologic malignancy, undiagnosed or relapsing hematologic malignancies should be considered in the differential diagnosis of sudden deaths.
Literatur
1.
Zurück zum Zitat Herishanu Y, Katz B-Z, Lipsky A, Wiestner A. Biology of chronic lymphocytic leukemia in different microenvironments: clinical and therapeutic implications. Hematol Oncol Clin North Am. 2013;27:173–206.CrossRef Herishanu Y, Katz B-Z, Lipsky A, Wiestner A. Biology of chronic lymphocytic leukemia in different microenvironments: clinical and therapeutic implications. Hematol Oncol Clin North Am. 2013;27:173–206.CrossRef
2.
Zurück zum Zitat Kipps TJ, Stevenson FK, Wu CJ, et al. Chronic lymphocytic leukaemia. Nat Rev Dis Primers. 2017;3:16096.CrossRef Kipps TJ, Stevenson FK, Wu CJ, et al. Chronic lymphocytic leukaemia. Nat Rev Dis Primers. 2017;3:16096.CrossRef
3.
Zurück zum Zitat Kipps TJ, Stevenson FK, Wu CJ, Croce CM, Packham G, Wierda WG, et al. Chronic lymphocytic leukaemia. Nat Rev Dis Primers. 2017;9:17008.CrossRef Kipps TJ, Stevenson FK, Wu CJ, Croce CM, Packham G, Wierda WG, et al. Chronic lymphocytic leukaemia. Nat Rev Dis Primers. 2017;9:17008.CrossRef
4.
Zurück zum Zitat Singh N, Singh Lubana S, Dabrowski L, Sidhu G. Leukostasis in chronic lymphocytic leukemia. Am J Case Rep. 2020;21:e924798. Singh N, Singh Lubana S, Dabrowski L, Sidhu G. Leukostasis in chronic lymphocytic leukemia. Am J Case Rep. 2020;21:e924798.
5.
Zurück zum Zitat Gong J, Wu B, Guo T, Zhou S, He B, Peng X. Hyperleukocytosis: A report of five cases and review of the literature. Oncol Lett. 2014;8:1825–7.CrossRef Gong J, Wu B, Guo T, Zhou S, He B, Peng X. Hyperleukocytosis: A report of five cases and review of the literature. Oncol Lett. 2014;8:1825–7.CrossRef
6.
Zurück zum Zitat Hölig K, Moog R. Leukocyte depletion by therapeutic leukocytapheresis in patients with leukemia. Transfus Med Hemother. 2012;39:241–5.CrossRef Hölig K, Moog R. Leukocyte depletion by therapeutic leukocytapheresis in patients with leukemia. Transfus Med Hemother. 2012;39:241–5.CrossRef
7.
Zurück zum Zitat Ali AM, Mirrakhimov AE, Abboud CN, Cashen AF. Leukostasis in adult acute hyperleukocytic leukemia: a clinician’s digest: Leukostasis: a clinician’s digest. Hematol Oncol. 2016;34:69–78.CrossRef Ali AM, Mirrakhimov AE, Abboud CN, Cashen AF. Leukostasis in adult acute hyperleukocytic leukemia: a clinician’s digest: Leukostasis: a clinician’s digest. Hematol Oncol. 2016;34:69–78.CrossRef
8.
Zurück zum Zitat Stucki A, Rivier AS, Gikic M, Monai N, Schapira M, Spertini O. Endothelial cell activation by myeloblasts: molecular mechanisms of leukostasis and leukemic cell dissemination. Blood. 2001;97:2121–9.CrossRef Stucki A, Rivier AS, Gikic M, Monai N, Schapira M, Spertini O. Endothelial cell activation by myeloblasts: molecular mechanisms of leukostasis and leukemic cell dissemination. Blood. 2001;97:2121–9.CrossRef
9.
Zurück zum Zitat Barbier V, Erbani J, Fiveash C, Davies JM, Tay J, Tallack MR, et al. Endothelial E-selectin inhibition improves acute myeloid leukaemia therapy by disrupting vascular niche-mediated chemoresistance. Nat Commun. 2020;11:2042.CrossRef Barbier V, Erbani J, Fiveash C, Davies JM, Tay J, Tallack MR, et al. Endothelial E-selectin inhibition improves acute myeloid leukaemia therapy by disrupting vascular niche-mediated chemoresistance. Nat Commun. 2020;11:2042.CrossRef
10.
Zurück zum Zitat Würthner JU, Köhler G, Behringer D, Lindemann A, Mertelsmann R, Lübbert M. Leukostasis followed by hemorrhage complicating the initiation of chemotherapy in patients with acute myeloid leukemia and hyperleukocytosis: a clinicopathologic report of four cases. Cancer. 1999;85:368–74.CrossRef Würthner JU, Köhler G, Behringer D, Lindemann A, Mertelsmann R, Lübbert M. Leukostasis followed by hemorrhage complicating the initiation of chemotherapy in patients with acute myeloid leukemia and hyperleukocytosis: a clinicopathologic report of four cases. Cancer. 1999;85:368–74.CrossRef
11.
Zurück zum Zitat Rozovski U, Hazan-Halevy I, Barzilai M, Keating MJ, Estrov Z. Metabolism pathways in chronic lymphocytic leukemia. Leuk Lymphoma. 2016;57:758–65.CrossRef Rozovski U, Hazan-Halevy I, Barzilai M, Keating MJ, Estrov Z. Metabolism pathways in chronic lymphocytic leukemia. Leuk Lymphoma. 2016;57:758–65.CrossRef
12.
Zurück zum Zitat Lokich JJ, Moloney WC. Fatal pulmonary leukostasis following treatment in acute myelogenous leukemia. Arch Intern Med. 1972;130:759–62.CrossRef Lokich JJ, Moloney WC. Fatal pulmonary leukostasis following treatment in acute myelogenous leukemia. Arch Intern Med. 1972;130:759–62.CrossRef
13.
Zurück zum Zitat Bloom R, Taveira Da Silva AM, Bracey A. Reversible respiratory failure due to intravascular leukostasis in chronic myelogenous leukemia. Am J Med. 1979;67:679–83. Bloom R, Taveira Da Silva AM, Bracey A. Reversible respiratory failure due to intravascular leukostasis in chronic myelogenous leukemia. Am J Med. 1979;67:679–83.
14.
Zurück zum Zitat Megaludis AM, Winkelstein A, Zeigler ZR, Miller TR. Leukostasis: a phenomenon of prolymphocytic leukemia. Am J Hematol. 1989;32:146–7.CrossRef Megaludis AM, Winkelstein A, Zeigler ZR, Miller TR. Leukostasis: a phenomenon of prolymphocytic leukemia. Am J Hematol. 1989;32:146–7.CrossRef
15.
Zurück zum Zitat Ng MHL, Tsang SS, Ng HK, Sriskandavarman V, Feng CS. An unusual case of hairy cell leukemia: Death due to leukostasis and intracerebral hemorrhage. Hum Pathol. 1991;22:1298–302.CrossRef Ng MHL, Tsang SS, Ng HK, Sriskandavarman V, Feng CS. An unusual case of hairy cell leukemia: Death due to leukostasis and intracerebral hemorrhage. Hum Pathol. 1991;22:1298–302.CrossRef
16.
Zurück zum Zitat Beaubien ER, Wilson TW, Satkunam N. Sudden death in a patient with chronic lymphocytic leukemia. CMAJ. 1998;159:1123–5.PubMedPubMedCentral Beaubien ER, Wilson TW, Satkunam N. Sudden death in a patient with chronic lymphocytic leukemia. CMAJ. 1998;159:1123–5.PubMedPubMedCentral
17.
Zurück zum Zitat Stoecker MM, Wang E, Simmons S. Sudden death due to undiagnosed T lymphoblastic leukemia/lymphoma in a 5-year-old boy. Am J Forensic Med Pathol. 2012;33:314–6.CrossRef Stoecker MM, Wang E, Simmons S. Sudden death due to undiagnosed T lymphoblastic leukemia/lymphoma in a 5-year-old boy. Am J Forensic Med Pathol. 2012;33:314–6.CrossRef
18.
Zurück zum Zitat Gupta B, Arora P, Gupta R, Khurana N. T-acute lymphoblastic leukemia/lymphoma, an unexpected cause of sudden death. J Indian Acad Forensic Med. 2018;40:224.CrossRef Gupta B, Arora P, Gupta R, Khurana N. T-acute lymphoblastic leukemia/lymphoma, an unexpected cause of sudden death. J Indian Acad Forensic Med. 2018;40:224.CrossRef
19.
Zurück zum Zitat Patel AB, Pettijohn EM, Abedin SM, Raps E, Deininger MW. Leukemoid reaction in chronic myelomonocytic leukemia patients undergoing surgery: perioperative management recommendations. Blood Adv. 2019;3:952–5.CrossRef Patel AB, Pettijohn EM, Abedin SM, Raps E, Deininger MW. Leukemoid reaction in chronic myelomonocytic leukemia patients undergoing surgery: perioperative management recommendations. Blood Adv. 2019;3:952–5.CrossRef
20.
Zurück zum Zitat Harris AL. Leukostasis associated with blood transfusion in acute myeloid leukaemia. Br Med J. 1978;1:1169–71.CrossRef Harris AL. Leukostasis associated with blood transfusion in acute myeloid leukaemia. Br Med J. 1978;1:1169–71.CrossRef
21.
Zurück zum Zitat Abdel-Ghafar AA, El Din El Telbany MA, Mahmoud HM, El-Sakhawy YN. Immunophenotyping of chronic B-cell neoplasms: flow cytometry versus immunohistochemistry. Hematol Rep. 2012;4:e3. Abdel-Ghafar AA, El Din El Telbany MA, Mahmoud HM, El-Sakhawy YN. Immunophenotyping of chronic B-cell neoplasms: flow cytometry versus immunohistochemistry. Hematol Rep. 2012;4:e3.
22.
Zurück zum Zitat Babu SBS, Biradar G, Bakkannavar S, Kumar PG, Shaila B. Estimation of time since death from nuclei changes of bone marrow cells. Indian J Forensic Community Med. 2015;2:198.CrossRef Babu SBS, Biradar G, Bakkannavar S, Kumar PG, Shaila B. Estimation of time since death from nuclei changes of bone marrow cells. Indian J Forensic Community Med. 2015;2:198.CrossRef
23.
Zurück zum Zitat Larsson K, Mattsson M, Ebrahim F, Glimelius I, Höglund M. High prevalence and incidence of cardiovascular disease in chronic lymphocytic leukaemia: a nationwide population-based study. Br J Haematol. 2020;190:e245–8.CrossRef Larsson K, Mattsson M, Ebrahim F, Glimelius I, Höglund M. High prevalence and incidence of cardiovascular disease in chronic lymphocytic leukaemia: a nationwide population-based study. Br J Haematol. 2020;190:e245–8.CrossRef
24.
Zurück zum Zitat Cohen Y, Amir G, Da’as N, Gillis S, Rund D, Polliack A. Acute myocardial infarction as the presenting symptom of acute myeloblastic leukemia with extreme hyperleukocytosis. Am J Hematol. 2002;71:47–9.CrossRef Cohen Y, Amir G, Da’as N, Gillis S, Rund D, Polliack A. Acute myocardial infarction as the presenting symptom of acute myeloblastic leukemia with extreme hyperleukocytosis. Am J Hematol. 2002;71:47–9.CrossRef
25.
Zurück zum Zitat Htet ZM, Gomez C, Ali A, Nair S, Karim M. Multiple faces of chronic lymphocytic leukaemia: A patient with renal, cardiac, and skeletal complications. Case Report Nephrol. 2019;2019:1–7.CrossRef Htet ZM, Gomez C, Ali A, Nair S, Karim M. Multiple faces of chronic lymphocytic leukaemia: A patient with renal, cardiac, and skeletal complications. Case Report Nephrol. 2019;2019:1–7.CrossRef
26.
Zurück zum Zitat Lieberman F, Villgran V, Normolle D, Boyiadzis M. Intracranial hemorrhage in patients newly diagnosed with acute myeloid leukemia and hyperleukocytosis. Acta Haematol. 2017;138:116–8.CrossRef Lieberman F, Villgran V, Normolle D, Boyiadzis M. Intracranial hemorrhage in patients newly diagnosed with acute myeloid leukemia and hyperleukocytosis. Acta Haematol. 2017;138:116–8.CrossRef
Metadaten
Titel
Sudden death due to leukostasis in a subject with undiagnosed chronic lymphocytic leukemia
verfasst von
Lorenzo Gitto
Rohin Mehta
Kanish Mirchia
Christopher Ramos
Michel Tawil
Serenella Serinelli
Publikationsdatum
20.08.2021
Verlag
Springer US
Erschienen in
Forensic Science, Medicine and Pathology / Ausgabe 4/2021
Print ISSN: 1547-769X
Elektronische ISSN: 1556-2891
DOI
https://doi.org/10.1007/s12024-021-00406-9

Weitere Artikel der Ausgabe 4/2021

Forensic Science, Medicine and Pathology 4/2021 Zur Ausgabe

Neu im Fachgebiet Pathologie

Assistierter Suizid durch Infusion von Thiopental

Thiopental Originalie

Als Folge des Urteils des Bundesverfassungsgerichts zur Sterbehilfe im Jahr 2020 wurde in den Jahren 2021–2023 eine Reihe (n = 23) von assistierten Suiziden im Landesinstitut für gerichtliche und soziale Medizin Berlin mit jeweils identischen …

Molekularpathologische Untersuchungen im Wandel der Zeit

Open Access Biomarker Leitthema

Um auch an kleinen Gewebeproben zuverlässige und reproduzierbare Ergebnisse zu gewährleisten ist eine strenge Qualitätskontrolle in jedem Schritt des Arbeitsablaufs erforderlich. Eine nicht ordnungsgemäße Prüfung oder Behandlung des …

Vergleichende Pathologie in der onkologischen Forschung

Pathologie Leitthema

Die vergleichende experimentelle Pathologie („comparative experimental pathology“) ist ein Fachbereich an der Schnittstelle von Human- und Veterinärmedizin. Sie widmet sich der vergleichenden Erforschung von Gemeinsamkeiten und Unterschieden von …

Gastrointestinale Stromatumoren

Open Access GIST CME-Artikel

Gastrointestinale Stromatumoren (GIST) stellen seit über 20 Jahren ein Paradigma für die zielgerichtete Therapie mit Tyrosinkinaseinhibitoren dar. Eine elementare Voraussetzung für eine mögliche neoadjuvante oder adjuvante Behandlung bei …