B-Cell Acute Lymphocytic Leukemia Presenting With Secondary Hemophagocytic Lymphohistiocytosis as Disseminated Intravascular Coagulation and Shock Following Cardiac Arrest in an Adult

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can be familial in etiology or a result of infections, malignancy, and autoimmune or inflammatory disorders. Disseminated intravascular coagulation (DIC) is common in patients admitted to intensive care units and can confound and delay the diagnosis of HLH. We present a case of a 69-year-old female who presented with dyspnea and malaise. Her condition declined rapidly with laboratory parameters consistent with DIC. In addition, she had a ferritin of 32,522 ng/mL, low haptoglobin, and elevated LDH, and bone marrow biopsy showed hemophagocytic lymphohistiocytes. She was started on HLH-directed therapy, and later, a diagnosis of ALK-negative anaplastic large cell lymphoma was made on an excisional inguinal lymph node biopsy specimen. Our case emphasizes the importance of prompt recognition, diagnosis, and treatment of HLH while workup for a primary disorder is still being pursued.

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Results of Treatment With Hyper-CVAD, a Dose-Intensive Regimen, in Adult Acute Lymphocytic Leukemia

Journal of Clinical Oncology ◽

10.1200/jco.2000.18.3.547 ◽

2000 ◽

Vol 18 (3) ◽

pp. 547-547 ◽

Cited By ~ 537

Author(s):

Hagop M. Kantarjian ◽

Susan O’Brien ◽

Terry L. Smith ◽

Jorge Cortes ◽

Francis J. Giles ◽

...

Keyword(s):

B Cell ◽

Acute Lymphocytic Leukemia ◽

Philadelphia Chromosome ◽

Lymphocytic Leukemia ◽

Prognostic Models ◽

Poor Performance Status ◽

High Dose ◽

Cell Disease ◽

Adult Acute Lymphocytic Leukemia ◽

Intensive Regimen

PURPOSE: To evaluate the efficacy and toxicity of Hyper-CVAD (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone), a dose-intensive regimen, in adult acute lymphocytic leukemia (ALL). PATIENTS AND METHODS: Adults with newly diagnosed ALL referred since 1992 were entered onto the study; treatment was initiated in 204 patients between 1992 and January 1998. No exclusions were made because of older age, poor performance status, organ dysfunction, or active infection. Median age was 39.5 years; 37% were at least 50 years old. Mature B-cell disease (Burkitt type) was present in 9%, T-cell disease in 17%. Leukocytosis of more than 30 × 109/L was found in 26%, Philadelphia chromosome–positive disease in 16% (20% of patients with assessable metaphases), CNS leukemia at the time of diagnosis in 7%, and a mediastinal mass in 7%. Treatment consisted of four cycles of Hyper-CVAD alternating with four cycles of high-dose methotrexate (MTX) and cytarabine therapy, together with intrathecal CNS prophylaxis and supportive care with antibiotic prophylaxis and granulocyte colony-stimulating factor therapy. Maintenance in patients with nonmature B-cell ALL included 2 years of treatment with mercaptopurine, MTX, vincristine, and prednisone (POMP). RESULTS: Overall, 185 patients (91%) achieved complete remission (CR) and 12 (6%) died during induction therapy. Estimated 5-year survival and 5-year CR rates were 39% and 38%, respectively. The incidence of CNS relapse was low (4%). Compared with 222 patients treated with vincristine, doxorubicin, and dexamethasone (VAD) regimens, our patients had a better CR rate (91% v 75%, P < .01) and CR rate after one course (74% v 55%, P < .01) and better survival (P < .01), and a smaller percentage had more than 5% day 14 blasts (34% v 48%, P = .01). Previous prognostic models remained predictive for outcome with Hyper-CVAD therapy. CONCLUSION: Hyper-CVAD therapy is superior to our previous regimens and should be compared with established regimens in adult ALL.

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Disseminated Intravascular Coagulation and Malignancy: A Case Report and Literature Review

Case Reports in Oncological Medicine ◽

10.1155/2020/9147105 ◽

2020 ◽

Vol 2020 ◽

pp. 1-5

Author(s):

Sumit Sohal ◽

Akhilesh Thakur ◽

Aleena Zia ◽

Mina Sous ◽

Daniela Trelles

Keyword(s):

Atrial Fibrillation ◽

Pulmonary Embolism ◽

Cardiac Arrest ◽

Disseminated Intravascular Coagulation ◽

Limb Ischemia ◽

Symptomatic Treatment ◽

Malignant Cells ◽

Intravascular Coagulation ◽

Fluid Analysis ◽

History Of

Disseminated Intravascular Coagulation (DIC) is a disorder of coagulation which is commonly seen as a complication of infections, traumas, obstetric diseases, and cancers especially hematological and rarely solid cancers. DIC may rarely be the presenting feature of an undiagnosed malignancy. It may present in the form of different phenotypes which makes its diagnosis difficult and leads to high mortality. The treatment comprises supportive, symptomatic treatment and removal of the underlying source. Here, we present a patient with history of being on warfarin for atrial fibrillation and other comorbidities who presented with elevated INR of 6.3 and increasing dyspnea on exertion. Over the course of her stay, her platelet counts started dropping with a concurrent decrease in fibrinogen levels. She eventually developed pulmonary embolism, followed by stroke and limb ischemia, which was indicative of the thrombotic phenotype of DIC. Her pleural fluid analysis showed huge burden of malignant cells in glandular pattern suggestive of adenocarcinoma and was started on heparin drip. However, the patient had cardiac arrest and expired on the same day of diagnosis.

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B cell acute lymphocytic leukemia in adults. Clinical, morphologic, and immunologic findings.

Journal of Clinical Oncology ◽

10.1200/jco.1986.4.5.737 ◽

1986 ◽

Vol 4 (5) ◽

pp. 737-743 ◽

Cited By ~ 31

Author(s):

P S Gill ◽

P R Meyer ◽

Z Pavlova ◽

A M Levine

Keyword(s):

B Cell ◽

Acute Lymphocytic Leukemia ◽

Disease Free Survival ◽

Lymphocytic Leukemia ◽

Cell Phenotype ◽

History Of ◽

Acquired Immunodeficiency ◽

Immunologic Marker ◽

Immunodeficiency Syndrome

Acute lymphocytic leukemia (ALL) is a heterogeneous group of disorders, clinically, immunologically, and pathologically. ALL of a B cell phenotype (B-ALL) is the least common. We have studied ten adult patients with B-ALL, none of whom had a tumor mass. The median age was 56 years (range, 30 to 90). A history of an altered immune state was noted in four cases: a distant history of Hashimoto's thyroiditis in one, pregnancy in one, and acquired immunodeficiency syndrome in two. Two patients presented with CNS involvement, and in two additional patients CNS leukemia developed during the course of disease. By the French-American-British (FAB) classification system, L3 leukemic morphology was present in nine, whereas L2 was present in one. Circulating leukemic blasts varied from less than 500/dL to greater than 15,000/dL. Eight patients were thrombocytopenic, and eight were anemic at presentation. Immunologic marker studies on leukemic blasts revealed monoclonal kappa light chain marking in nine and monoclonal lambda in one. Following chemotherapy, complete remission was achieved in three patients, two of whom experienced relapse within 9 months. The median survival for the group was 3 months, and only one patient experienced long-term, disease-free survival. We conclude that B-ALL in the adult presents with the classic L3 morphologic picture in the majority and is associated with extremely short survival.

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Comparative Analysis of Systemic and Tumor Microenvironment Proteomes From Children With B-Cell Acute Lymphocytic Leukemia at Diagnosis and After Induction Treatment

Frontiers in Oncology ◽

10.3389/fonc.2020.550213 ◽

2020 ◽

Vol 10 ◽

Author(s):

Geise Ellen Broto ◽

Stephany Corrêa ◽

Fausto Celso Trigo ◽

Everton Cruz dos Santos ◽

Fernanda Tomiotto-Pelissier ◽

...

Keyword(s):

Transcription Factor ◽

B Cell ◽

Acute Lymphocytic Leukemia ◽

Lymphocytic Leukemia ◽

Induction Treatment ◽

Induction Phase ◽

Label Free ◽

Childhood Diseases ◽

Coagulation Proteins ◽

Ifn Γ

Among the childhood diseases, B-cell acute lymphocytic leukemia (B-ALL) is the most frequent type of cancer. Despite recent advances concerning disease treatment, cytotoxic chemotherapy remains the first line of treatment in several countries, and the modifications induced by such drugs in the organism are still poorly understood. In this context, the present study provided a comparative high-throughput proteomic analysis of the cumulative changes induced by chemotherapeutic drugs used in the induction phase of B-ALL treatment in both peripheral blood (PB) and bone marrow compartment (BM) samples. To reach this goal, PB and BM plasma samples were comparatively analyzed by using label-free proteomics at two endpoints: at diagnosis (D0) and the end of the cumulative induction phase treatment (D28). Proteomic data was available via ProteomeXchange with identifier PXD021584. The resulting differentially expressed proteins were explored by bioinformatics approaches aiming to identify the main gene ontology processes, pathways, and transcription factors altered by chemotherapy, as well as to understand B-ALL biology in each compartment at D0. At D0, PB was characterized as a pro-inflammatory environment, with the involvement of several downregulated coagulation proteins as KNG, plasmin, and plasminogen. D28 was characterized predominantly by immune response-related processes and the super expression of the transcription factor IRF3 and transthyretin. RUNX1 was pointed out as a common transcription factor found in both D0 and D28. We chose to validate the proteins transthyretin and interferon-gamma (IFN-γ) by commercial kits and expressed the results as PB/BM ratios. Transthyretin ratio was augmented after induction chemotherapy, while IFN-γ was reduced at the end of the treatment. Considering that most of these proteins were not yet described in B-ALL literature, these findings added to understanding disease biology at diagnosis and highlighted a possible role for transthyretin and IFN-γ as mechanisms related to disease resolution.

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Abstract 3262: Targeting PI3K-coupled MEK/ERK pathway for therapy in pediatric B-cell acute lymphocytic leukemia.

10.1158/1538-7445.am2013-3262 ◽

2013 ◽

Author(s):

Xiang Wang ◽

Ben-shang Li ◽

Li-xia Ding ◽

Chris Liang ◽

Jian Ding ◽

...

Keyword(s):

B Cell ◽

Acute Lymphocytic Leukemia ◽

Lymphocytic Leukemia ◽

Erk Pathway

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Case 17: Severe bleeding complications, lymphocytosis and leukoerythroblastosis – disseminated intravascular coagulation in a patient with metastasizing carcinoma of the prostate and chronic lymphocytic leukemia

Therapeutische Umschau ◽

10.1024/0040-5930.56.9.533 ◽

1999 ◽

Vol 56 (9) ◽

pp. 533-536 ◽

Cited By ~ 1

Author(s):

Solenthaler ◽

Lämmle

Keyword(s):

Chronic Lymphocytic Leukemia ◽

Disseminated Intravascular Coagulation ◽

Lymphocytic Leukemia ◽

Bleeding Complications ◽

Severe Bleeding ◽

Disseminierte Intravasale Gerinnung ◽

Intravascular Coagulation ◽

Carcinoma Of The Prostate

Blutungskomplikationen bei chronischer lymphatischer Leukämie (CLL) stehen meist in Zusammenhang mit einer Thrombozytopenie, bedingt entweder durch eine direkte Verdrängung der Megakaryopoese bei diffuser Knochenmarksinfiltration oder durch einen vermehrten peripheren Verbrauch im Rahmen einer (sekundären) Immunthrombozytopenie. Eine disseminierte intravasale Gerinnung (DIC) gehört nicht zum Spektrum hämatologischer Komplikationen einer CLL. Das hier geschilderte Fallbeispiel eines Patienten mit schweren Blutungskomplikationen, labormäßigen Zeichen einer DIC und neu entdeckter CLL ließ eine Ursache der DIC vermissen. Das leukoerythroblastäre Blutbild lieferte den Schlüssel zur Diagnose eines metastasierenden Prostatakarzinoms, welches durch die Knochenmarksbiopsie bestätigt wurde und als Trigger für die DIC verantwortlich war.

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