
==== Front
Cureus
Cureus
2168-8184
Cureus
2168-8184
Cureus Palo Alto (CA)

10.7759/cureus.67744
Dermatology
Oncology
Adult-Onset T-Cell Acute Lymphoblastic Lymphoma-Leukemia Presenting With Petechial Rash: A Case Report
Muacevic Alexander
Adler John R
Kimball Kelly 1
Elad Vissy 2
Hamad Edward J 2
Wasco Christopher 1
1 Internal Medicine, OhioHealth Riverside Methodist Hospital, Columbus, USA
2 College of Medicine, Northeast Ohio Medical University, Rootstown, USA
Kelly Kimball kelly.kimball@ohiohealth.com
25 8 2024
8 2024
16 8 e6774425 8 2024
Copyright © 2024, Kimball et al.
2024
Kimball et al.
https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
This article is available from https://www.cureus.com/articles/252206-adult-onset-t-cell-acute-lymphoblastic-lymphoma-leukemia-presenting-with-petechial-rash-a-case-report
T-cell acute lymphoblastic lymphoma-leukemia (T-ALL) is a rare neoplastic disease with presenting symptoms that are often non-specific. As such, accurate diagnosis requires high clinical suspicion and assessment of bone marrow aspirate with flow cytometry and morphology. While remission is achievable in most patients, the five-year survival rate is only 48% despite treatment. The standard chemotherapy regimen is referred to as hyper-CVAD, where CVAD stands for cyclophosphamide, vincristine sulfate, Adriamycin (doxorubicin), methotrexate, and dexamethasone. In this study, we describe a 38-year-old female who presented with atraumatic abdominal bruising and a petechial rash on the upper and lower extremities. Imaging revealed a 16 cm anterior mediastinal mass and a bone marrow and mediastinal mass biopsy confirmed a diagnosis of T-ALL. The patient received hyper-CVAD cycle 1A with several complications but ultimately achieved remission after four cycles. Here, we stress the importance of broadening differentials for new-onset petechial rashes in adults to include systematic lymphomas, such as T-ALL, and the need for early recognition so patients can receive timely treatment.

oncology
dermatology
chemotherapy
petechial rash
t-cell lymphoma
==== Body
pmcIntroduction

T-cell acute lymphoblastic lymphoma-leukemia (T-ALL) is a rare neoplastic disease with an incidence of 4,000 cases in the United States each year [1]. The majority of cases appear in childhood, between the ages of two and 10 years. Within the adult population, this cancer is even rarer, with T-ALL accounting for approximately 25% of all cases of ALL in adults [2]. A retrospective population-based analysis of the Surveillance, Epidemiology, and End Results Program (SEER) database between 2001 and 2014 showed the incidence of T-ALL in the United States adult population to be 0.13 cases per 100,000 [3]. Initial presentations of T-ALL are non-specific and include symptoms, such as weight loss, weakness, lymphadenopathy, easy bruising, and skin pallor [1]. Diagnosis involves the assessment of bone marrow aspirate and its subsequent flow cytometry, morphology, and above all, adequate clinical suspicion. The main immunophenotype markers in T-ALL include terminal deoxynucleotidyl transferase (TdT) and CD3, with other markers, such as CD1a, CD2, CD4, CD5, CD7, and CD8, being variably expressed [4]. To achieve remission of T-ALL, patients are typically treated with multiagent intrathecal and intravenous chemotherapy delivered in intensive courses over two to three years with a possibility for adjunct post-remission radiation therapy in cases with CNS involvement [5]. One prospective trial of 334 patients found that 94% of T-ALL patients achieved complete remission after 2-phase induction over eight weeks and showed a five-year overall survival rate of 48%; patients were followed for 10 years [6,7]. Many patients with T-ALL receive treatment with a regimen commonly referred to as hyper-CVAD, where CVAD stands for cyclophosphamide, vincristine sulfate, Adriamycin (doxorubicin), methotrexate, and dexamethasone [4].

In this report, we describe a 38-year-old female who presented with atraumatic abdominal bruising and a petechial rash on the upper and lower extremities. She was found to have T-ALL and a 16 cm anterior mediastinal mass. Herein, we stress the importance of broadening differentials for new-onset petechial rashes in otherwise healthy adults to include systemic diseases, such as hematologic malignancies.

Case presentation

A 38-year-old female with no significant medical history presented with a six-day history of progressive atraumatic abdominal bruising and petechial rash on her upper and lower extremities in October 2023 (Figures 1, 2). These symptoms were accompanied by drenching night sweats, low-grade fevers, chest heaviness, and heavy menstruation.

Figure 1 Multiple, atraumatic, non-blanching petechiae involving the upper extremities.

Figure 2 Generalized petechial eruption with non-palpable purpura and ecchymoses involving the bilateral lower extremities.

Laboratory studies showed a white blood cell count of 42,000/μL and a platelet count of 8,000/μL (Table 1). A computed tomography (CT) pulmonary embolism study was negative for pulmonary embolism but remarkable for a large soft tissue mass in the anterior mediastinum measuring up to 16 cm (Figure 3).

Table 1 Abnormal patient laboratory findings at presentation.

Test	Patient values	Reference range	
White blood cells (count/μL)	42,000	4,500-11,000	
Platelet count (count/μL)	8,000	150,000-450,000	
Lactate dehydrogenase (U/L)	>2,500	140-280	
Serum uric acid (mg/dL)	13.4	2.7-7.3	
Serum fibrinogen (mg/dL)	493	200-400	

Figure 3 A homogeneous anterior mediastinal mass with local mass effect measuring 16 cm (arrows).

Core needle biopsy of the mediastinal mass demonstrated diffuse effacement of the biopsy by immature mononuclear cells, compatible with lymphoblasts. By immunohistochemistry analysis, the neoplastic cells had strong nuclear staining with TdT, were positive for CD99, weakly positive for CD5, and positive for CD3 in a pattern consistent with T-ALL. Initial bone marrow biopsy of the iliac crest showed effacement with lymphoblasts positive for strong nuclear TdT and cytoplasmic CD3 as well as CD99 and CD1a, consistent with T-ALL. There was an absence of significant keratin staining with a keratin AE1/AE3 immunostain, which is again consistent with T-ALL. A potential diagnosis of acute T-cell leukemia/lymphoma (ATLL) was considered due to the increased number of circulating lymphoblasts in the peripheral blood smear. However, this diagnosis was ruled out based on negative testing for human T-lymphotropic virus types 1 and 2 (HTLV-1/2) infection (Figure 4). She ultimately received the diagnosis of adult-onset T-ALL given her bone marrow biopsy results. The patient received hyper-CVAD cycle 1A which includes cyclophosphamide, vincristine, doxorubicin, and dexamethasone. Her hospitalization was complicated by subdural hemorrhage, acute kidney injury, vaginal bleeding, and mucositis. She was discharged home in stable condition 19 days later; however, she did have some lingering sinus congestion and voice hoarseness.

Figure 4 Peripheral blood smear with increased circulating lymphoblasts (10×).

The patient presented to the hospital two days after discharge with complaints of gland swelling, epistaxis, ear soreness, and ongoing sinus congestion and eventually tested positive for parainfluenza 2. She was admitted and treated with intravenous (IV) ampicillin-sulbactam, and then later treated with IV vancomycin. She went on to receive cycle 1B of hyper-CVAD five days later, which included methotrexate and cytarabine and was tolerated well. She received intrathecal (IT) chemotherapy on day two with methotrexate as prophylaxis for leptomeningeal spread. She was discharged home receiving day seven (D7) IT chemotherapy with cytarabine three days later as an outpatient. Cerebrospinal fluid was negative for malignant cells. She recovered well with symptomatic management and did not require readmission. After four cycles of hyper-CVAD, a bone marrow biopsy of the iliac crest was obtained for restaging of her disease; however, the aspirate did not show any T-lymphoblasts identified via flow cytometry, supporting the attainment of molecular remission of her T-ALL. Positron emission tomography (PET)-CT restaging 1.5 months later showed almost complete resolution of her mediastinal mass; therefore, consolidative radiation was not pursued. The patient remains in remission and is receiving two years of prednisone, vincristine, methotrexate, and mercaptopurine (POMP) maintenance therapy with pegylated (PEG) asparaginase intensification.

Discussion

Here, we present an innocuous case of T-ALL with a constellation of non-specific findings. We stress the importance of broadening differentials for new-onset petechial rashes in adults to include systematic lymphomas, such as T-ALL, and highlight the need for early recognition so patients can receive timely treatment. The patient received hyper-CVAD cycle 1A and had several complications, but ultimately achieved remission after four cycles. This case is a strong example of how high clinical suspicion for relatively rare etiologies of disease can reduce patient morbidity and mortality. The median overall survival of T-ALL in adult populations is estimated to be 34 months; one of the potential contributing factors to this statistic is delay/interruption of therapy, such as early relapse, treatment-related toxicity, and non-compliance [3]. In cases such as these, subtle dermatologic findings like this bear the potential to play a major role in elucidating the final diagnosis [7,8]. While the lymph nodes and mediastinum are sites most frequently implicated in T-ALL, other sites like the skin or kidneys should not be subject to a lower threshold of diagnostic scrutiny due to their lower frequency of involvement in this genre of neoplastic processes [9]. It is estimated that between 5% and 8% of all visits to the emergency department are due to dermatologic concerns, and while petechiae and purpura can often be non-specific skin findings, they should not be overlooked by providers [10]. In patients with T-ALL who develop petechial rashes, the legs are the most commonly involved location for lesions to develop, which aligns with this patient’s presentation [11]. Interestingly, one study examined over 5,000 patients with ALL and found that spleen and liver enlargement are prevalent in T-ALL, findings that were not observed in the present case [12]. This may be attributed to the patient’s age, as these findings become less frequent in older populations, possibly due to age-related atrophy of lymphoid organs [12]. There have been other instances where a patient’s presenting symptoms do not immediately appear to be the result of a hematologic malignancy. For example, the case of a 19-year-old male with T-cell lymphoblastic lymphoma who presented with acute renal failure, lactic acidosis, and a very near normal complete blood count, highlighting the diverse presentations of T-ALL and the risk for misdiagnosis [13]. Cases like these serve as reminders to consider more than the most probable diagnosis and reinforce the value of thorough investigation. In an otherwise healthy person, a chief complaint of petechial/purpuric rash warrants further workup to diagnose potential systemic diseases, such as hematologic malignancies. Fortunately for this patient, she was able to achieve molecular remission and tumor regression, likely due to early identification and treatment.

Conclusions

This study highlights several key take-home points, with one of the most notable being the importance of having a broad differential for adult-onset petechial rashes. These differentials may include T-ALL, which often presents with non-specific findings and needs a high clinical suspicion for diagnosis. In otherwise healthy individuals, the onset of a new petechial/purpuric rash provides a rationale for further investigation to diagnose potential systemic diseases, including hematologic malignancies. The sooner a systemic work-up is started by the healthcare team, the greater the likelihood of successful clinical outcomes, as was experienced in the present case.

The authors would like to thank Dr. Charles Nicely for his contribution to obtaining the hematopathology slides for this case.

Disclosures

Author Contributions

Human subjects: Consent was obtained or waived by all participants in this study.

Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following:

Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work.

Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work.

Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Concept and design:  Edward J. Hamad, Kelly Kimball, Vissy Elad, Christopher Wasco

Drafting of the manuscript:  Edward J. Hamad, Kelly Kimball, Vissy Elad, Christopher Wasco

Critical review of the manuscript for important intellectual content:  Edward J. Hamad, Kelly Kimball, Vissy Elad, Christopher Wasco

Acquisition, analysis, or interpretation of data:  Kelly Kimball, Christopher Wasco

Supervision:  Kelly Kimball, Christopher Wasco
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References

1 T-cell acute lymphoblastic leukemia Hematology Am Soc Hematol Educ Program Raetz EA Teachey DT 580 588 2016 2016 27913532
2 Acute leukemia incidence and patient survival among children and adults in the United States, 2001-2007 Blood Dores GM Devesa SS Curtis RE Linet MS Morton LM 34 43 119 2012 22086414
3 Incidence and survival of T-cell acute lymphoblastic leukemia in the United States Leuk Lymphoma Murthy GS Pondaiah SK Abedin S Atallah E 1171 1178 60 2019 30407885
4 Management of adults with T-cell lymphoblastic leukemia Blood Marks DI Rowntree C 1134 1142 129 2017 28115371
5 Central nervous system involvement in adult acute lymphoblastic leukemia at diagnosis: results from the international ALL trial MRC UKALL XII/ECOG E2993 Blood Lazarus HM Richards SM Chopra R 465 472 108 2006 16556888
6 Induction therapy for adults with acute lymphoblastic leukemia: results of more than 1500 patients from the international ALL trial: MRC UKALL XII/ECOG E2993 Blood Rowe JM Buck G Burnett AK 3760 3767 106 2005 16105981
7 T-cell acute lymphoblastic leukemia in adults: clinical features, immunophenotype, cytogenetics, and outcome from the large randomized prospective trial (UKALL XII/ECOG 2993) Blood Marks DI Paietta EM Moorman AV 5136 5145 114 2009 19828704
8 Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology. Eighth Edition Clinical Dermatology, 8e. McGraw-Hill Education Wolff K Johnson R Saavedra AP Roh EK New York City, NY McGraw-Hill Education 2017 https://accessmedicine.mhmedical.com/content.aspx?bookid=2043&sectionid=154893575
9 Precursor B-cell lymphoblastic lymphoma: a predominantly extranodal tumor with low propensity for leukemic involvement Am J Surg Pathol Lin P Jones D Dorfman DM Medeiros LJ 1480 1490 24 2000 11075849
10 Cutaneous conditions leading to dermatology consultations in the emergency department West J Emerg Med Jack AR Spence AA Nichols BJ Chong S Williams DT Swadron SP Peng DH 551 555 12 2011 22224158
11 Adult T-cell acute lymphoblastic leukemia presenting as concurrent viral exanthem-like reaction and palpable purpura Kaohsiung J Med Sci Yang YT Lin FL 311 312 39 2023 36727927
12 Clinico-biological features of 5202 patients with acute lymphoblastic leukemia enrolled in the Italian AIEOP and GIMEMA protocols and stratified in age cohorts Haematologica Chiaretti S Vitale A Cazzaniga G 1702 1710 98 2013 23716539
13 Acute renal failure and type B lactic acidosis as first manifestation of extranodal T-cell lymphoblastic lymphoma BMJ Case Rep Yun S Walker CN Vincelette ND Anwer F 2014 2014
