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Wednesday, 30 June 2021

BURKITT LYMPHOMA: DIAGNOSIS AT A GLANCE IN A CLINICAL LABORATORY

Written by Ana Belén García Ruano | Juan Francisco Gutiérrez Bautista | Irene Romero García

Figure 1. A: Blood differential. B. Scattergram (Sysmex XN-1000) C: Alarm of blast cells. D: Maygrünwald-Giemsa stain of peripheral blood.

A 74-year-old patient arrives at the the emergency room with dysphagia, intolerance to solids and liquids, that has been evolving for 3 to 4 days, nausea, constipation and a feeling of abdominal distension. Blood chemistry tests and complete blood count (CBC) arrives at the emergency laboratory. The CBC results showed leukocytosis (52.59 x 103/μL), at the expense of monocytes and lymphocytes, thrombopenia and alarm by blast cells (Figure 1A). The lymphocyte and monocyte populations can’t be properly separated in the scattergram, and the population seems to be only made up of a single cellular type (Figure 1B). Furthermore, in the WPC scattergramm the blast cells are the principal component of this population (Figure 1C). A peripheral blood cytomorphology stained with May-Grünwald-Giemsa is performed. We observed that approximately 80% of the leucocytes are medium-sized cells, with round nuclei and immature appearance. They also present scarce cytoplasm and an intense polar vacuolization. This characteristic finding makes us suspect a potential Burkitt Lymphoma (Figure 1D).
The biochemical tests showed an acute kidney failure (creatinine 4.47 mg/dL), along with high levels of LDH (9694 U/mL). Additional determinations of uric acid, magnesium and phosphorus are carried out, confirming a tumour lysis syndrome, with hyperuricemia (30 mg/dL), hypermagnesemia and hyperphosphatemia. Along with the data supplied by the morphology, acute lymphoid hemopathy is suspected, suggesting acute lymphoblastic leukaemia L3.

The last classification of the World Health Organisation (WHO) in 2016, does not only use morphologic criteria and clinical characteristics to settle on the diagnostic of haematological neoplasia. Thus, an immunophenotyping study is carried out, as well as, a haematological phenotyping, both coming from bone marrow (Figure 2). The pathological population that, makes up 40% of the cellular total, presents the following phenotype: CD45++ CD19+ CD23het CD10+ CD5- CD56- CD20++ IgsLambda+ CD22dim CD43- CD200- FMC7+ CD81+ ROR1- CD79b+ CD44- CD38+ CD305- DR+ CD123- CD27- CD11c+ +CD79b+ CD103- CD25+ (Figure 2A). This immunophenotypic study is compatible with mature neoplasia B cells, suggesting a Burkitt leukaemia. None the less, we await for the genetic confirmation with the karyotype, where we are able to see a juxtaposition of the genes IGH and MYC, t(8;14)(q24;q32); what defines cytogenetic feature of Burkitt's lymphoma (Figure 2B).

imagen2Figure 2. A: Immunophenotype of the bone marrow. B: Katyotype of the bone marrow.

The WHO classifies Burkitt's lymphoma in B cells in three types: endemic, sporadic and associated with immunosuppression. This type of leukaemia owes its name to Burkitt, who first described it in 1958 in middle African children. In Western countries, the sporadic subtype represents between 1-2% of lymphomas in adults. It also appears related to immunodeficiencies. At the time of diagnosis, most of the patients, as in our case, present medullary, abdominal and extra-ganglionic involvement.

The significance of this clinical case resides in how, from within the multidisciplinary emergency laboratory, we have been able to, regardless of the low prevalence of the sporadic Burkitt lymphoma, guide the clinician towards a diagnostic thanks to aset of images (Figure 1) and some biochemical data. This started a cascade of studies that concluded in an early diagnosis of Burkitt's lymphoma.

REFERENCE

  1. Khalil Saleh, Jean-Marie Michot , Valérie Camara-Clayette, Yegor Vassetsky, Vincent Ribrag. Burkitt and Burkitt-Like Lymphomas: a Systematic Review. Curr Oncol Rep. 2020 Mar 6;22(4):33. doi: 10.1007/s11912-020-0898-8.
  2. Sapkota S, Shaikh H. Non-Hodgkin Lymphoma. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan. 2020 Aug 10.
  3. Mata Fernández, C. Linfoma de Burkitt: el tumor pediátrico más frecuente en África / Burkitt's lymphoma. The most common paediatric tumour in Africa. Acta pediatr. esp;66(7):322-326, jul. 2008. ilus.
  4. Steven H. Swerdlow, Elias Campo, Stefano A. Pileri, Nancy Lee Harris, Harald Stein, Reiner Siebert, Ranjana Advani, Michele Ghielmini, Gilles A. Salles, Andrew D. Zelenetz, and Elaine S. Jaffe. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016 May 19; 127(20): 2375–2390. Prepublished online 2016 Mar 15. doi: 10.1182/blood-2016-01-643569.
  5. Swerdlow, S.H., Campo, E., Pileri, S.A., Harris, N.L., Stein, H., Siebert, R., Advani, R., Ghielmini, M., Salles, G.A., Zelenetz, A.D. & Jaffe, E.S. (2016) The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, 127, 2375–2390

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