Hairy Cell Leukemia Presenting Research Articles

Simultaneous Presentation of Hairy Cell Leukemia and Acute Lymphoblastic Leukemia

Simultaneous Presentation of Hairy Cell Leukemia and Acute Lymphoblastic Leukemia

Simultaneous Presentation of Hairy Cell Leukemia and Acute Lymphoblastic Leukemia

Simultaneous Presentation of Hairy Cell Leukemia and Acute Lymphoblastic Leukemia

Skeletal Lesions and Adductor Compartment Mass: A Rare Presentation of Hairy Cell Leukemia

Skeletal Lesions and Adductor Compartment Mass: A Rare Presentation of Hairy Cell Leukemia

Outcome of Atypical Presentations of Hairy Cell Leukemia

Hairy cell leukemia (HCL) is a distinctchronic lymphoproliferative disorder that accounts for 2% of all adult leukemias. The median age at the time of diagnosis is 52 years with a male to female ratio of 4:1. Hairy cell leukemia predominantlycharacterized by infiltration of peripheral blood, bone marrow, and spleen by malignant cells with prominent irregular cytoplasmic projections, namely, "hairy cells". HCL patients may be asymptomatic or develop symptoms of cytopenia, particularly infections . The exact prevalence of atypical presentation of HCL is difficult to be determined because most cases were published as incidental reports. The classical presentation of Hairy cell leukaemia includes splenomegaly (80-90%), pancytopenia (60-80%) and hepatomegaly (40-50%), however, the same may not be true in the clinical settings. As hairy cell leukemia may present with a wide range of atypical organ involvements which could result in a diagnostic dilemma. The aim of this literature review is to highlight non- classical presentations of HCL and the importance to have high index of suspicion to consider a diagnosis of HCL based on histomorphological features even in the absence of classical clinical features. Method: We searched the medline database (PubMed), GoogleScholar from1985 until 2018 with the following search terms: atypical presentation of hairy cell leukemia ,unusual cases of hairy cell leukemia,unique hairy cell leukemia presentation. Result: A total of 21 cases were found, with age range from (29-88),5 cases were female and the rest were males. Skeletal involvement was the most common presentation with osteolytic bone lesion. Majority of the patients were treated with Cladribine,13/21 showed complete remission. Discussion: HCL is an indolent disorder of B-cell origin that accounts for 2% of all forms of leukemia and 8% of lymphoproliferative diseases. In our literature review we looked up the unique presentations, which Most of the reported cases we found involved the skeleton in the form of pathological fracture and osteolytic lesion. Serositis, in the form of massive ascites and pleural effusion has been reported. Additionally, Cases of central nervous system involvement and presentation at young age were observed. Due to rarity of the disease and no established treatment modality for unusual organ involvement, standard treatment with Cladribine result in complete remission in 61% of the observed cases. Conclusion: While Classical presentation of hairy cell leukemia is well described in the literature. atypical presentation is still rare but with diverse presentation. most of the cases reviewed in the literature were middle age males, majority had good response to treatment, while the rest had disease progression . rare pretension of Hairy cell leukemia despite being rare should always be considered ,particularly in cases of pathological fractures or unexplained serositis. Table Disclosures No relevant conflicts of interest to declare.

Hairy Cell Leukemia Presenting as Presternal Soft Tissue Mass: A Case Report With Review of Literature

Abstract Hairy cell leukemia (HCL) is an uncommon chronic lymphoproliferative disorder. Initial presentation frequently includes symptoms related to peripheral blood cytopenias. It can sometimes have atypical manifestations and can present at unusual sites. We report an unusual presentation of HCL as a presternal soft tissue mass in a 45-year-old man. His peripheral blood counts showed pancytopenia. There were no palpable lymph nodes or hepatosplenomegaly. CT-scan revealed a middle mediastinal mass with extension into the presternal soft tissue. Tissue core biopsy was taken and histopathologic findings confirmed the diagnosis of HCL. To our knowledge, this is the second reported case of HCL presenting as a presternal soft tissue mass. Although rare, HCL should be considered in the differential diagnosis of tumors involving extramedullary/extranodal sites including the soft tissue and bone, so the patient can get the utmost benefit for early diagnosis of a treatment responsive disease.

Simultaneous presentation of plasma cell myeloma and hairy cell leukemia

Simultaneous presentation of plasma cell myeloma and hairy cell leukemia

Hairy cell leukemia presenting with Ecthyma Gangrenosum- a case report

BackgroundEcthyma gangrenosum is a cutaneous infectious usually associated with P. aeruginosa. It usually develops In patients with an underlying immunodeficiency.Case presentationA 50-year old mentally disabled white male with a history of epilepsy presented with fever and a painless red macule on his right arm which rapidly progressed to a painful ulcer. Blood and lesion cultures revealed P.aeruginosa, confirming our clinical diagnosis of ecthyma gangrenosum. Subsequently an underlying immune deficit was found, namely patient was diagnosed with hairy-cell leukemia. Despite adequate antibiotics no infection control could be achieved. After treating the underlying immune deficit as well, the infection and hairy-cell leukemia resolved completely.ConclusionEcthyma gangrenosum is an important cutaneous infection to recognize, because it is it is typically associated with P.aeruginosa bacteremia. Recognizing this skin leasion should prompt empiric antimicrobial therapy including an agent with antipseudomonal activity. Furthermore, just like in our case, the presence of ecthyma gangrenosum can signal the presence of an occult immune deficit, warranting further investigation.

Primary lymphomatous presentation of hairy cell leukemia as osteolytic vertebral lesions: a case report

Hairy cell leukemia (HCL) is a rare and indolent mature B cell neoplasm. Most patients with HCL have excellent response to purine analogs and BRAF inhibitors. Therefore, it is clinically important to identify and differentiate HCL from other B cell neoplasms. HCL is predominantly found in the peripheral blood, bone marrow, or spleen. HCL presenting initially or solely in unusual sites is extremely rare (approximately ten or fewer). We hereby report a unique case of HCL presenting as vertebral osteolytic and epidural mass lesions without bone marrow, spleen, or peripheral blood involvement. The patient was a 53-year-old male with acute and chronic radicular pain down the posterior aspect of bilateral thighs. Imaging study with MRI demonstrated a L4 posterior vertebral osteolytic mass lesion, extending into the spinal canal, and a second right sacral S1 lesion extending into the sacral iliac joint. These two noncontiguous lesions were thought to be consistent with metastatic cancer or sarcoma, and the specimen was submitted to non-hematopoietic pathologist for diagnosis without flow cytometry. Initial immunohistochemical stains were ordered for solid tumor markers, which were all negative. As part of the workup, immunostain for BRAF V600E (VE1) was ordered for melanoma, and CD138 and cyclin D1 for myeloma, which returned positive and suggested the possibility of hairy cell leukemia. Additional workup for hairy cell leukemia confirmed the diagnosis. HCL typically presents with peripheral blood, bone marrow, or spleen involvement, while mass lesions in other sites are rare. High index of suspicion is essential for the correct diagnosis of HCL in mass lesions at unusual sites. In the absence of initial hematopathological workup and flow cytometry, HCL presenting as solid mass in unusual locations is misdiagnosed most likely as marginal zone B cell lymphoma (mucosa-associated lymphoid tissue (MALT) lymphoma). The expression of cyclin D1, TRAP, annexin A1, and BRAF V600E mutation would confirm the diagnosis of HCL. The main differentials include MALT lymphoma, mantle cell lymphoma (MCL), and hairy cell leukemia variant (HCLv). This case report will be interesting for the clinicians and pathologists alike and broaden the spectrum of clinical presentation of lymphomatous HCL.

İzole Kemik Lezyonları ile Ortaya Çıkan Saçlı Hücreli Lösemi

İzole Kemik Lezyonları ile Ortaya Çıkan Saçlı Hücreli Lösemi

Hairy Cell Leukemia Presenting with a Lytic Bone Lesion

Hairy cell leukemia (HCL) is an indolent neoplasm of small mature B lymphoid cells with characteristic morphologic features usually involving the peripheral blood, bone marrow, and spleen. It constitutes approximately 2% of adult leukemias and has a male predominance. Patients usually present with weakness, splenomegaly, and pancytopenia. The overall prognosis is favorable with appropriate treatment including purine analogs, interferon-alpha, and radiation. We report a patient with a history of breast cancer who presented with a left femoral lytic bone lesion that was subsequently diagnosed as HCL by morphology, immunohistochemistry, flow cytometry, and molecular genetic techniques. It was important to biopsy the lesion to establish the correct diagnosis, as HCL is a highly treatment-responsive malignancy.

Hairy Cell Leukemia Presenting with Isolated Skeletal Involvement Successfully Treated by Radiation Therapy and Cladribine: A Case Report and Review of the Literature.

We describe an unusual case of hairy cell leukemia (HCL) in a 55-year-old male presenting with isolated skeletal disease as the initial manifestation without abnormal peripheral blood counts, bone marrow involvement, or splenomegaly. To the best of our knowledge, there have been only two previous reports of a similar case. The patient presented with pain in the right femur. Anteroposterior radiographs of both femurs revealed mixed lytic-sclerotic lesions. PET scan showed multiple metastatic lesions on axial skeleton, pelvis, and both femurs. Histopathological examination of the bone biopsy revealed an infiltrate of HCL. Localized radiation therapy to both proximal femurs and subsequently 4 weeks later, a 7-day course of 0.1 mg/kg/day cladribine provided complete remission with relief of symptoms and resolution of bone lesions. We addressed the manifestations and management of HCL patients with skeletal involvement.

Hairy cell leukemia presenting as a palpable breast mass

Hairy cell leukemia (HCL) is a rare mature B cell malignancy that typically involves the bone marrow, peripheral blood, and spleen. Clinically detectable lymph node infiltration occurs in a minority of patients, and extranodal tissue involvement of sites such as the central nervous system, gastrointestinal tract, and skin is uncommon. Consequently, the vast majority of patients with HCL present with pancytopenia and splenomegaly but without lymphadenopathy or soft tissue masses. Herein we present a unique case of HCL presenting as a palpable breast mass without concomitant marrow or peripheral blood involvement and without radiologic evidence of other sites of disease. Several features of the tumor cell infiltrate (ample pale cytoplasm, nested appearance) evoked other entities that would more typically involve breast tissue such as the alveolar or solid variants of invasive lobular carcinoma. However, the immunophenotypic and molecular characteristics of the tumor cells were indistinguishable from classic HCL. The significant challenge in this case was recognizing this entity outside of its usual clinical and anatomic context. Although rare, HCL should be a diagnostic consideration in tumors arising at extramedullary/extranodal sites including the breast.

A case report of hairy cell leukemia presenting concomitantly with sweet syndrome.

Hairy cell leukemia and Sweet syndrome are both uncommon hematological diagnoses. We present a patient who was admitted with fevers, pancytopenia, pneumonia, and rash. Diagnostic bone marrow biopsy demonstrates Hairy cell Leukemia and skin biopsy demonstrates neutrophils infiltration consistent with Sweet syndrome. The patient was treated with purine analogs with resolution of the cytopenias, infection, and rash.

Hairy-Cell Leukemia Presenting As Lytic Bone Lesions

Hairy-Cell Leukemia Presenting As Lytic Bone Lesions

Simultaneous Presentation of Hairy Cell Leukemia and Metastatic Signet Ring Carcinoma of the Stomach Diagnosed by Bone Marrow Biopsy

Hairy cell leukemia (HCL) is a rare chronic B-cell disorder with an increased risk of second tumors. The relative risk of second cancers reported in various series of HCL patients ranged from 0.95 to 4.33, but simultaneous presentation of HCL and other epithelial malignancies is very rare. To our knowledge, we present the first case of the coexistence of signet ring carcinoma of the stomach and HCL. We report a case of the simultaneous presentation of HCL and metastasis of the primary signet ring cell carcinoma of the stomach to the bone marrow and skin. A bone marrow biopsy was performed on a patient with pancytopenia. A histologic examination with immunostaining of the bone marrow specimen showed the presence of signet ring carcinoma cells in addition to HCL. As a consequence, our patient underwent further diagnostic procedures including endo-gastro-duodenoscopy with biopsy of gastric tumor mass, which established the diagnosis of gastric signet ring cell carcinoma.

Hairy cell leukemia presenting as a cranial mass

A 42-year-old gentleman presented with a one month history of occipital swelling, on a background of intermittent headaches over the previous 6 months. He was, otherwise, fit and well. He had suffered a head injury 2 years earlier but there was no other past medical history of note, and he took no regular medications. His family history revealed a sibling and an aunt to have had Hodgkin’s lymphoma. He was a nonsmoker and drank 21 units of alcohol per week. Examination confirmed a right-sided occipital swelling but was otherwise unremarkable. Imaging of the head was performed using both computed tomography (Image 1) and magnetic resonance imaging (Image 2). A solid lesion was seen to overlie the right occipital bone with permeative destruction of the underlying skull vault and intracranial extension into the extradural space. Biopsy of the lesion was performed under ultrasound guidance. Histology revealed monomorphic intermediate sized cells expressing CD20, CD79a, BCL2 and cyclin D1, suggestive of a B cell lymphoid neoplasm. A full blood count demonstrated bicytopenia (white blood cell count 1.7 3 10/L haemoglobin 14.5 g/dL, platelet count 74 3 10/L), with the unusual finding of monocytopenia (0.1 3 10/L). Bone marrow aspiration revealed a population of cells morphologically resembling hairy cell leukemia (Image 3). Flow cytometry confirmed this diagnosis, revealing a population of B cells with the classical immunophenotypic profile for hairy cell leukemia: CD20, CD22, CD11c, CD25, CD103, and CD123. Positron emission tomography-computed tomography of the chest, abdomen, and pelvis revealed disseminated disease, with splenomegaly, widespread lymphadenopathy Image 2. Coronal T2-weighted magnetic resonance imaging scan of the head, revealing intracranial extension of the lesion into the extradural space. Image 1. Axial computed tomography scan of the head, demonstrating a solid mass lesion with destruction of the underlying skull vault.

Visual Disturbance as Initial Presentation of Hairy Cell Leukemia

Hairy cell leukemia (HCL) is a rare disorder that occasionally has visual symptoms after diagnosis. The authors present a case of HCL in which bilateral visual symptoms led to the initial diagnosis. Observational case report. Bilateral decreased vision to 20/30 in the right eye and 20/40 in the left due to intraretinal and preretinal hemorrhages with no other systemic signs or symptoms prompted a hematologic evaluation in which HCL was found to be the causative disorder in a previously healthy 41-year-old man. The authors present a rare case in which bilateral visual complaints led to the diagnosis of HCL. Thus, though uncommon, HCL should be considered in the differential diagnosis of otherwise unexplained retinal hemorrhages.

Hairy cell leukemia presenting as multiple discrete hepatic lesions

The involvement of hairy cell leukemia in the liver is in the form of portal and sinusoidal cellular infiltration. Here we describe the first case of hepatic hairy cell leukemia presenting as multiple discrete lesions, which was treated successfully. We suggest that in the investigation of discrete hepatic lesions in cases of cancer of unknown primary, hairy cell leukemia should be considered. The excellent response of hairy cell leukemia to therapy highlights the need for such a consideration.

Extranodal hairy cell leukemia presenting in the lumbar spine

The authors report on a 54-year-old man who presented with a lumbar vertebral body lesion and an adjacent epidural lesion that was found to be hairy cell leukemia (HCL). The patient presented with gradual onset of back pain and intermittent lower-extremity radicular symptoms. He did not have splenomegaly or peripheral blood count abnormalities. Admission MR imaging revealed an L-5 vertebral body lesion and a lumbar epidural lesion extending from L-3 to S-2. An [18F]fluorodeoxyglucose-PET study showed numerous sites of osseous involvement. The patient underwent minimally invasive surgical biopsy sampling of the epidural lesion. Histopathological examination revealed extranodal HCL. After treatment with a 5-day course of cladribine, the patient's symptoms resolved, and at the 16-week follow-up visit there was no radiographic or metabolic evidence of disease. Hairy cell leukemia rarely involves neurological structures, but this patient responded well to standard treatment. This case demonstrates the value of tissue biopsy procedures instead of aggressive resection and the use of minimally invasive techniques to treat an HCL spinal lesion.

Hairy cell leukemia presenting as a discrete liver mass: Diagnosis by fine needle aspiration biopsy

Hairy cell leukemia, a chronic low-grade lymphoproliferative disorder first recognized in mid 1920's typically involves the lymphoreticular system, especially the spleen and the bone marrow. Clinically, it tends to present with pancytopenia and splenomegaly. Liver involvement, if present, is usually in a sinusoidal and a periportal pattern. We report a case of hairy cell leukemia associated with a liver mass 20 years after the initial presentation. To our knowledge, this is the first case of hairy cell leukemia presenting as a solid liver mass diagnosed by a percutaneous ultrasound guided fine needle aspiration biopsy to be reported in the English literature.