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01-06-2021 | Subdural Hematoma | Case Report

Juvenile myelomonocytic leukemia presenting in an infant with a subdural hematoma

Authors: William A. Lambert, Joseph A. DiGiuseppe, Tatiana Lara-Ospina, Markus J. Bookland, Jonathan E. Martin, David S. Hersh

Published in: Child's Nervous System | Issue 6/2021

Abstract

Background

Juvenile myelomonocytic leukemia (JMML) is a rare childhood hematopoietic disorder typically presenting with hepatosplenomegaly, lymphadenopathy, pallor, fever, and cutaneous findings. The authors report the first case, to our knowledge, of JMML presenting in a pediatric patient with a subdural hematoma.

Case description

A 7-month old male with recurrent respiratory infections and a low-grade fever presented with a full fontanelle and an increasing head circumference and was found to have chronic bilateral subdural collections. Abusive head trauma, infectious, and coagulopathy workups were unremarkable, and the patient underwent bilateral burr holes for evacuation of the subdural collections. The postoperative course was complicated by the development of thrombocytopenia, anemia, and an acute subdural hemorrhage which required evacuation. Cytologic analysis of the subdural fluid demonstrated atypical cells, which prompted flow cytometric analysis, a bone marrow biopsy, and ultimately a diagnosis of JMML. Following chemotherapy, the patient’s counts improved, and he subsequently underwent a hematopoietic stem cell transplant.

Conclusion

Subdural collections may rarely represent the first presenting sign of hematologic malignancies. In patients with a history of recurrent infections and a negative workup for abusive head trauma, clinicians should include neoplastic etiologies in the differential for chronic subdural collections and have a low threshold for fluid analysis.

Abdulhamid MM, Li YM, Hall WA (2011) Spontaneous acute subdural hematoma as the initial manifestation of chronic myeloid leukemia. J Neuro-Oncol 101(3):513–516. https://doi.org/10.1007/s11060-010-0278-6CrossRef

Basmaci M, Hasturk AE (2012) Chronic subdural hematoma in a child with acute myeloid leukemia after leukocytosis. Indian J Crit Care Med 16(4):222–224. https://doi.org/10.4103/0972-5229.106508CrossRefPubMedPubMedCentral

Board, P. D. Q. P. T. E (2002) Childhood acute myeloid leukemia/other myeloid malignancies treatment (PDQ®): health professional version. In: PDQ Cancer Information Summaries. National Cancer Institute (US), Bethesda

Bromberg JE, Vandertop WP, Jansen GH (1998) Recurrent subdural haematoma as the primary and sole manifestation of chronic lymphocytic leukaemia. Br J Neurosurg 12(4):373–376. https://doi.org/10.1080/02688699844934CrossRefPubMed

Chan RJ, Cooper T, Kratz CP, Weiss B, Loh ML (2009) Juvenile myelomonocytic leukemia: a report from the 2nd International JMML Symposium. Leuk Res 33(3):355–362. https://doi.org/10.1016/j.leukres.2008.08.022CrossRefPubMed

Choudhary AK, Servaes S, Slovis TL, Palusci VJ, Hedlund GL, Narang SK, Moreno JA, Dias MS, Christian CW, Nelson MD Jr, Silvera VM, Palasis S, Raissaki M, Rossi A, Offiah AC (2018) Consensus statement on abusive head trauma in infants and young children. Pediatr Radiol 48(8):1048–1065. https://doi.org/10.1007/s00247-018-4149-1CrossRefPubMed

Comănescu A, Roşca E, Bota M, Ninulescu G (2008) Chronic subdural hematoma in a patient with acute myeloid leukemia and dural metastatic infiltration. Romanian J Morphol Embryol 49(2):259–262

Cseh A, Niemeyer CM, Yoshimi A, Dworzak M, Hasle H, van den Heuvel-Eibrink MM, Locatelli F, Masetti R, Schmugge M, Groß-Wieltsch U, Candás A, Kulozik AE, Olcay L, Suttorp M, Furlan I, Strahm B, Flotho C (2015) Bridging to transplant with azacitidine in juvenile myelomonocytic leukemia: a retrospective analysis of the EWOG-MDS study group. Blood 125(14):2311–2313. https://doi.org/10.1182/blood-2015-01-619734CrossRefPubMed

Flotho C (2019) Gene mutations do not operate in a vacuum: the increasing importance of epigenetics in juvenile myelomonocytic leukemia. Epigenetics 14(3):236–244. https://doi.org/10.1080/15592294.2019.1583039CrossRefPubMedPubMedCentral

10.

Hamdy N, Bokhary H, Elsayed A, Hozayn W, Soliman S, Salem S, Alsheshtawi K, Abdalla A, Hafez H, Hammad M (2020) RAS pathway mutation patterns in patients with juvenile myelomonocytic leukemia: a developing country single-center experience. Clin Lymphoma Myeloma Leuk 20(7):e368–e374. https://doi.org/10.1016/j.clml.2020.02.003CrossRefPubMed

11.

Iversen PO, Rodwell RL, Pitcher L, Taylor KM, Lopez AF (1996) Inhibition of proliferation and induction of apoptosis in juvenile myelomonocytic leukemic cells by the granulocyte-macrophage colony-stimulating factor analogue E21R. Blood 88(7):2634–2639CrossRef

12.

Jourdan E, Dombret H, Glaisner S, Micléa JM, Castaigne S, Degos L (1995) Unexpected high incidence of intracranial subdural haematoma during intensive chemotherapy for acute myeloid leukaemia with a monoblastic component. Br J Haematol 89(3):527–530. https://doi.org/10.1111/j.1365-2141.1995.tb08358.xCrossRefPubMed

13.

Kitano A, Yamamoto K, Nagao T, Yoshida M, Kurosu T, Arai A, Miki T, Murakami N, Miura O (2005) Successful treatment with drainage of hematoma and chemotherapy in a case of Burkitt leukemia presenting with subdural hematoma. Rinsho Ketsueki 46(4):278–280PubMed

14.

Koike K, Matsuda K (2008) Recent advances in the pathogenesis and management of juvenile myelomonocytic leukaemia. Br J Haematol 141(5):567–575. https://doi.org/10.1111/j.1365-2141.2008.07104.xCrossRefPubMed

15.

Kratz CP, Niemeyer CM (2005) Juvenile myelomonocytic leukemia. Hematology 10(Suppl 1):100–103. https://doi.org/10.1080/10245330512331390078CrossRefPubMed

16.

Lasho T, Patnaik MM (2020) Juvenile myelomonocytic leukemia - a bona fide RASopathy syndrome. Best Pract Res Clin Haematol 33(2):101171. https://doi.org/10.1016/j.beha.2020.101171CrossRefPubMed

17.

Lin CH, Hung GY, Chang CY, Chien JC (2005) Subdural hemorrhage in a child with acute promyelocytic leukemia presenting as subtle headache. J Chin Med Assoc 68(9):437–440. https://doi.org/10.1016/s1726-4901(09)70161-3CrossRefPubMed

18.

Locatelli F, Niemeyer CM (2015) How I treat juvenile myelomonocytic leukemia. Blood 125(7):1083–1090. https://doi.org/10.1182/blood-2014-08-550483CrossRefPubMed

19.

Locatelli F, Algeri M, Merli P, Strocchio L (2018) Novel approaches to diagnosis and treatment of Juvenile Myelomonocytic Leukemia. Expert Rev Hematol 11(2):129–143. https://doi.org/10.1080/17474086.2018.1421937CrossRefPubMed

20.

Marcu A, Colita A, Radu LE, Jercan CG, Bica AM, Asan M, Coriu D, Tanase AD, Diaconu CC, Mambet C, Botezatu A, Pasca S, Teodorescu P, Anton G, Gurban P, Colita A (2020) Single-center experience with epigenetic treatment for juvenile myelomonocytic leukemia. Front Oncol 10:484. https://doi.org/10.3389/fonc.2020.00484CrossRefPubMedPubMedCentral

21.

Niemeyer CM, Flotho C (2019) Juvenile myelomonocytic leukemia: who's the driver at the wheel? Blood 133(10):1060–1070. https://doi.org/10.1182/blood-2018-11-844688CrossRefPubMed

22.

Niemeyer CM, Arico M, Basso G, Biondi A, Cantu Rajnoldi A, Creutzig U, Haas O, Harbott J, Hasle H, Kerndrup G, Locatelli F, Mann G, Stollmann-Gibbels B, van't Veer-Korthof E, van Wering E, Zimmermann M (1997) Chronic myelomonocytic leukemia in childhood: a retrospective analysis of 110 cases. European Working Group on Myelodysplastic Syndromes in Childhood (EWOG-MDS). Blood 89(10):3534–3543PubMed

23.

Olk-Batz C, Poetsch AR, Nöllke P, Claus R, Zucknick M, Sandrock I, Witte T, Strahm B, Hasle H, Zecca M, Starý J, Bergstraesser E, de Moerloose B, Trebo M, van den Heuvel-Eibrink MM, Wojcik D, Locatelli F, Plass C, Niemeyer CM, Flotho C, the European Working Group of Myelodysplastic Syndromes in Childhood (EWOG-MDS) (2011) Aberrant DNA methylation characterizes juvenile myelomonocytic leukemia with poor outcome. Blood 117(18):4871–4880. https://doi.org/10.1182/blood-2010-08-298968CrossRefPubMed

24.

Palma JA, Dominguez PD, Riverol M (2013) Intracranial extramedullary hematopoiesis associated with multiple myeloma. Neurology 80(17):1620. https://doi.org/10.1212/WNL.0b013e31828f1899CrossRefPubMed

25.

Paulus S, Koronowska S, Fölster-Holst R (2017) Association between juvenile myelomonocytic leukemia, juvenile xanthogranulomas and neurofibromatosis type 1: case report and review of the literature. Pediatr Dermatol 34(2):114–118. https://doi.org/10.1111/pde.13064CrossRefPubMed

26.

Pitner SE, Johnson WW (1973) Chronic subdural hematoma in childhood acute leukemia. Cancer 32(1):185–190. https://doi.org/10.1002/1097-0142(197307)32:1<185::aid-cncr2820320128>3.0.co;2-oCrossRefPubMed

27.

Porto L, Kieslich M, Schwabe D, Zanella FE, Lanfermann H (2004) Central nervous system imaging in childhood leukaemia. Eur J Cancer 40(14):2082–2090. https://doi.org/10.1016/j.ejca.2004.04.024CrossRefPubMed

28.

Prasad BC, Chandra VV, Varaprasad G (2012) Dural metastases in chronic myeloid leukemia presenting as subdural hematoma. Turk Neurosurg 22(6):777–778. https://doi.org/10.5137/1019-5149.Jtn.4050-10.2CrossRefPubMed

29.

Sakaguchi H, Okuno Y, Muramatsu H, Yoshida K, Shiraishi Y, Takahashi M, Kon A, Sanada M, Chiba K, Tanaka H, Makishima H, Wang X, Xu Y, Doisaki S, Hama A, Nakanishi K, Takahashi Y, Yoshida N, Maciejewski JP, Miyano S, Ogawa S, Kojima S (2013) Exome sequencing identifies secondary mutations of SETBP1 and JAK3 in juvenile myelomonocytic leukemia. Nat Genet 45(8):937–941. https://doi.org/10.1038/ng.2698CrossRefPubMed

30.

Wajid MA, Gupta AK, Das G, Sahoo D, Meena JP, Seth R (2020) Outcomes of juvenile myelomonocytic leukemia patients after sequential therapy with cytarabine and 6-mercaptopurine. Pediatr Hematol Oncol 37(7):573–581. https://doi.org/10.1080/08880018.2020.1767244CrossRef

Title: Juvenile myelomonocytic leukemia presenting in an infant with a subdural hematoma
Authors: William A. Lambert
Joseph A. DiGiuseppe
Tatiana Lara-Ospina
Markus J. Bookland
Jonathan E. Martin
David S. Hersh
Publication date: 01-06-2021
Publisher: Springer Berlin Heidelberg
Keywords: Subdural Hematoma
Abusive Head Injury
Hematoma
Thrombocytopenia
Neurofibromatosis Type 1
Leukemia
Published in: Child's Nervous System / Issue 6/2021
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-020-05013-7

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Juvenile myelomonocytic leukemia presenting in an infant with a subdural hematoma

Abstract

Background

Case description

Conclusion

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Background

Case description

Conclusion

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