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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 10  |  Issue : 1  |  Page : 29-33

Hyperoeosinophilia at diagnosis in adolescent acute lymphoblastic leukaemia/lymphoma: A case report and review of the literature


1 Department of Clinical Hematology and Bone Marrow Transplant, Tata Medical Center, Kolkata, India
2 Faculty of Medicine and Defence Health, Universiti Pertahanan National Malaysia (National Defence University of Malaysia), Kem Perdana Sungai Besi, Kuala Lumpur, Malaysia

Date of Submission08-Sep-2019
Date of Acceptance13-Nov-2019
Date of Web Publication03-Jan-2020

Correspondence Address:
Mainul Haque
Faculty of Medicine and Defence Health, Universiti Pertahanan National Malaysia (National Defence University of Malaysia), Kem Perdana Sungai Besi, 57000 Kuala Lumpur
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AIHB.AIHB_102_19

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  Abstract 


Acute lymphoblastic leukaemia (ALL) or lymphoblastic lymphoma (LBL) presenting as peripheral blood hyperoeosinophilia is a sporadic disorder with fewer than fifty cases described since 1973, and the cumulative incidence is <1%. ALL/LBL with hyperoeosinophilia and the absence of blasts in peripheral blood might lead to misdiagnosis of ALL/LBL. The physician should keep in mind that oeosinophilia can be part of acute leukaemia and should be carefully investigated. In this case report, we discuss a case of B cell ALL/LBL (B-ALL/B-LBL) with peripheral blood hyperoeosinophilia and in the absence of blasts in peripheral blood. A 17-year-old young boy presented with low-grade fever, itching, bilateral progressive limb weakness and pleural effusion. His bone marrow and paravertebral mass were diagnostic of B-ALL/B-LBL. Around half of the patients with ALL associated with hyperoeosinophilia show cytogenetic abnormalities and most common anomalies are t (5;14) (q31; q32), which is otherwise rare in ALL, but in the current case report, has normal karyotype.

Keywords: Acute lymphoblastic leukaemia, hyperoeosinophilia, lymphoblastic lymphoma


How to cite this article:
Jahan D, Haque M. Hyperoeosinophilia at diagnosis in adolescent acute lymphoblastic leukaemia/lymphoma: A case report and review of the literature. Adv Hum Biol 2020;10:29-33

How to cite this URL:
Jahan D, Haque M. Hyperoeosinophilia at diagnosis in adolescent acute lymphoblastic leukaemia/lymphoma: A case report and review of the literature. Adv Hum Biol [serial online] 2020 [cited 2022 Oct 3];10:29-33. Available from: https://www.aihbonline.com/text.asp?2020/10/1/29/275083




  Introduction Top


Oeosinophils generally found only 1%–3% of the differential count of peripheral white blood cells, with normal range of 350–500 cells/mm 3 of blood.[1],[2] The severity of oeosinophilia divided into mild, (absolute oeosinophil count [AEC] 500–1500/mm 3), moderate (AEC 1500–5000/mm 3) and severe (AEC >5000/mm 3).[2],[3],[4] Hypersensitive disorders; parasitic infections; pure genetic transmissible form and haemato-oncologic diseases such as Hodgkin's lymphoma, chronic myeloid leukaemia, acute lymphoblastic leukaemia (ALL), chronic myeloid leukaemia and pernicious anaemia may present as severe oeosinophilia.[1],[2],[4] Lymphoblastic lymphoma (LBL) is a haematologic cancer of immature B cells obligated to the B or T-cell lineage and accounts for around 2% of all lymphomas.[5],[6],[7],[8],[9] There is a biological and clinical overlay between LBL and ALL, and LBL and ALL were almost similar types of haematological cancer with diverse clinical signs and symptoms.[5],[10] Both ALL or LBL usually present with fever, lymphadenopathy, hepatosplenomegaly and presence of lymphoblasts in the peripheral blood and bone marrow.[11],[12] ALL or LBL and hyperoeosinophilia represent a distinct clinicopathological entity,[13],[14] although it is rare in adolescent and young adults [15],[16],[17] with 44 cases reported to date.[18] The presenting symptoms are non-specific high fever, itching, progressive bilateral lower limb weakness, and hyperoeosinophilia has been considered as a reactive rather than a leukaemic reactions,[1],[14] were related with shoddier scenario, both in children [17] and adults.[19] B-ALL/LBL accounts for 2% of lymphoid cancers detected in the USA.[20] The incidence of B-ALL in the USA and other countries in both paediatric and adult population is increasing day by day.[5],[20],[21],[22],[23],[24],[25] B-cell acute lymphoproliferative disorders denoted as pure leukaemia (B-ALL) in 80% of incidence, isolated extramedullary disease (B-LBL) in 10% and B-ALL/B-LBL in 10% of incidence.[20] Extramedullary signs and symptoms when appear at the time of diagnosis determine and represent bone marrow involvement and often morphologically manifest or require identification by high-resolution flow cytometry.[20]


  Case Report Top


A 17-year-old male presented dated August 17, 2016 (Patient ID: Apollo UHID 630068) in Apollo Hospitals Dhaka (Plot: 81, Block: E, Bashundhara R/A, Dhaka 1229, Bangladesh) with fever and itching, low back pain, neck pain, bilateral mild pleural effusion, urinary incontinence and progressive bilateral lower limb weakness for 3 weeks. There was no history of allergies, skin rash or parasitic infestation. On neurological examination, the Glasgow Coma Scale found E4V5M6, and he had no lymphadenopathy or organomegaly except bilateral pleural effusion. The magnetic resonance imaging of the whole spine showed a space-occupying lesion at epidural, opposite dorsal and sacral (D4-D8, and S1) vertebrae and dorsal cord compression at D5-D8 levels [Figure 1]. Patient's the laboratory and biochemical data on admission depicted in [Table 1] and [Table 2]. He underwent urgent decompressive laminectomy and debunking of the tumour mass, and high-dose steroid was given, which made the diagnosis difficult. High-dose steroid might reduce the blast percentage in bone marrow, and it was revealed a 20% blast when we do bone marrow aspiration after surgery. Peripheral blood film revealed high leukocyte count (26.34 × 109) with oeosinophil constituting 43.8%; AEC was 11. 53 × 109. No atypical cells or haemoparasites were seen [Figure 2]. Bone marrow aspiration morphology showed cellular marrow with immature cells admixed with oeosinophils [Figure 3]. Flow cytometry from bone marrow revealed blast cells 20%. Blast cells were positive for CD45, CD10, CD19, CD79a, CD34, human leukocyte antigen – DR isotype and terminal deoxynucleotidyl transferase (TdT) and negative for MPO, CD13, CD33 [Figure 4] and immunohistochemistry from vertebral lesion and paravertebral mass positive for PAX5, TdT, CD79a, CD34 and CD45 and negative for Bcl6, CD5, CD7, CD20, MPO and Ki-67 was 40%. The bone marrow cytogenetic study was normal male pattern [Figure 5]. Computed tomography scan of the chest exhibited bilateral pleural effusion probably due to oeosinophilic infiltration in the lungs and pleural cavity. Echocardiogram with the left ventricular ejection fraction of 55% and cerebrospinal fluid study was negative for malignant cells. The patient was relocated to the haematology ward for chemotherapy. Started BFM-95 pediatric inspired chemoprotocol. The condition of the patient improved after chemotherapy and trailed up for 2 years, and right now, the patient is effectively alleviated.
Figure 1: Compressing dorsal spine by paravertebral mass.

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Table 1: Laboratory data on admission

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Table 2: Biochemical data on admission

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Figure 2: Peripheral blood containing oeosinophils with no blast cells.

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Figure 3: Bone marrow reveals oeosinophils and lymphoblast.

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Figure 4: Eight color flow cytometry showing acute lymphoblastic leukemia cluster differentiation markers.

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Figure 5: Cytogenetic study shows normal male Karyotype (46, XY).

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  Discussion Top


The current patient had severe oeosinophilia. Oeosinophilia connected with ALL was first reported by Spitzer and Garson in 1973.[26] Two cases of ALL with oeosinophilia were reported by Parasole et al.[13] The median age at diagnosis is 14 years; male preponderance and the majority are B-cell origins. Patients regularly present with signs and symptoms of organ involvement due to infiltration of organs with oeosinophils. Heart, lungs, skin, central nervous system, liver and spleen are usually involved. Cough, shortness of breath, cardiomegaly and lung infiltrates often observed in one-third to two-thirds of patients.[19] Cytogenetic abnormalities are detected about half of the patients with ALL associated with hyperoeosinophilia.[19] The most common cytogenetic foible observed in patients with ALL related to oeosinophilia is t (5;14) (q31; q32).[15] The oeosinophilia is followed by overproduction of interleukin (IL)-3, IL-5 and granulocyte-stimulating factor by the blasts due to instigation of growth factor gene on chromosome 5 when it is translocated head-to-head to the immunoglobulin heavy chain gene on the chromosome.[2],[19] The chromosomal irregularity is typically seen only in leukaemic blasts, and the oeosinophils had normal karyotype in 90% of cases supportive of the hypothesis that the oeosinophilia is reactive.[16] The current patient had a normal karyotype. The current patient presented with severe oeosinophilia and organ involvement like skin rash and mild bilateral pleural effusion probably due to oeosinophilic infiltration. Oeosinophils secrete oeosinophil granulated proteins which are accountable for the cardiotoxic effects such as acute pericarditis, myocarditis or endocarditis, and the development of thrombi adjacent to the injured myocardium and succeeding fibrosis lead to restrictive heart failure.[17] One earlier study reported that 61-year-old male presented with signs of oeosinophilic toxicity including myocardial infarction, oeosinophilic pneumonia and urticaria subsequently was diagnosed to have B ALL.[18] Another study revealed that two patients with oeosinophilia related to ALL also developed myocardial infarction, respiratory failure and cerebrovascular accident as severe difficulties of oeosinophilia.[14] The prognosis of patients with ALL connected with hyperoeosinophilia is poor with a median survival of 7.5 months.[2] In a review of ALL associated with hyperoeosinophilia, 26% of deaths were oeosinophilia related.[19] Since anthracyclines are an essential component of chemotherapy for ALL, oeosinophilic infiltration of the heart and lungs could compromise the treatment. Oeosinophilia in the peripheral blood smear of patients with ALL/LBL has been reported more often than other types of leukaemia.[19] Most recently, Wilson and Tefferi [14] reported two cases of pre-B cell ALL that initially presented with prolonged oeosinophilia and respiratory distress. After a period, a diagnosis of leukaemia was made. Oeosinophilia usually leads to the diagnosis of ALL and rapidly resolves upon induction, but it typically returns with leukaemia reversion. The prognosis for ALL and hyperoeosinophilia is expressively shoddier than for ALL alone. In some reports, oeosinophilia preceded the ALL diagnosis by 1–9 months.[14],[27] Follows et al. reported on a 43-year old patient with 13,400/mm 3 oeosinophils in peripheral blood smear, who had a normal platelet count and haemoglobin level and subsequently developed migratory arthritis with periarticular soft-tissue swellings and hepatosplenomegaly.[28] This patient was finally diagnosed as an ALL case. In general, reported patients with significant oeosinophilia and ALL are adults.[28] However, Files et al.[29] reported on an 8-year-old male with hyperoeosinophilia and Loeffler endocarditis who was diagnosed with ALL after 3 months. In comparison to the standard definition of ALL, congestive heart failure is the leading cause of augmented mortality in patients with ALL with hyperoeosinophilia.[30] A literature review shows that among ALL cases, there is a sturdy male great in number, a median age of 14 years (range 2–58) at presentation, and most examples are B-cell in origin.[30] Multiple studies revealed that a major portion of the patients with ALL related with hyperoeosinophilia shows cytogenetic abnormalities and most common anomalies are t (5;14) (q31; q32), which is otherwise rare in ALL, but in the current patient, has normal karyotype.[19],[31],[32],[33]


  Conclusion Top


Hyperoeosinophilia with ALL/LBL at diagnosis is very rare, particularly the absence of blast in peripheral blood might delay the diagnosis. Rarely, ALL/LBL is detected by oeosinophilia presenting with temperately amplified white blood cell and a higher percentage of oeosinophils. Hence, in patients with consistent hyperoeosinophilia with no influential reactive causes, bone marrow investigation is a must for an accurate diagnosis. It has been estimated of ALL/LBL presenting as hyperoeosinophilia is pointedly worse than ALL alone. Consequently, these patients need immediate diagnosis and intensive therapy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's father has given his consent for images and other clinical information to be reported in the journal. The patient's father understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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