Extramedullary Myeloid Tumor Research Articles

Sustained HIV remission after allogeneic hematopoietic stem cell transplantation with wild-type CCR5 donor cells.

HIV cure has been reported for five individuals who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) with cells from CCR5Δ32 homozygous donors. By contrast, viral rebound has occurred in other people living with HIV who interrupted antiretroviral treatment after undergoing allo-HSCT, with cells mostly from wild-type CCR5 donors. Here we report the case of a male individual who has achieved durable HIV remission following allo-HSCT with cells from an unrelated HLA-matched (9 of 10 matching for HLA-A, HLA-B, HLA-C, HLA-DRB1 and HLA-DQB1 alleles) wild-type CCR5 donor to treat an extramedullary myeloid tumor. To date, plasma viral load has remained undetectable for 32 months after the interruption of antiretroviral treatment. Treatment with ruxolitinib has been maintained during this period to treat chronic graft-versus-host disease. Low levels of proviral DNA were detected sporadically after allo-HSCT, including defective but not intact HIV DNA. No virus could be amplified in cultures of CD4+ T cells obtained after antiretroviral treatment interruption, while CD4+ T cells remained susceptible to HIV-1 infection in vitro. Declines in HIV antibodies and undetectable HIV-specific T cell responses further corroborate the absence of viral rebound after antiretroviral treatment interruption. These results suggest that HIV remission could be achieved in the context of allo-HSCT with wild-type CCR5.

Myeloid Sarcoma Presenting as Rectosigmoid Tumour: A Case Report

Myeloid Sarcoma (MS) or extramedullary myeloid tumor is a rare tumor affecting the skin, lymph node, and any part of the body except the bone marrow. MS may present differently depending on the site of involvement. A 61-year-old male with a known case of chronic myeloid leukemia presented to the emergency department of a district hospital with per rectal bleeding for two days. Examination revealed haematochezia with no evidence of rectal tumor. Hyperleukocytosis was noted from the blood investigation. Colonoscopy was performed, which revealed the rectosigmoid tumor. Histopathological examination confirmed the diagnosis of myeloid sarcoma. The patient was subsequently referred to a hematologist in a tertiary hospital for further care. MS may present a diagnostic challenge as it may present nonspecific symptoms depending on the site of involvement. Awareness of this condition is essential; thus, diagnosis and treatment can be initiated earlier.

Circulating Megakaryocytes in Peripheral Blood: A Clue to Satisfactory Platelet Recovery Post Induction Chemotherapy in Acute Myeloid Leukemia

Thrombocytopenia is a common hematological abnormality in newly diagnosed acute myeloid leukemia (AML) which may be linked to the degree of marrow infiltration by leukemic blasts as well as altered cytokine milieu in a subset of AMLs. We report the presence of circulating megakaryocytes in peripheral blood as a sign of satisfactory platelet recovery post induction chemotherapy in a pediatric subject with a favourable risk WHO category I AML with synchronous extramedullary myeloid cell tumor; and present a brief review of literature on the role of platelet recovery in AML prognosis.

Monoblastic Sarcoma- A Rare Case Report of Myeloid Sarcoma Variant

Myeloid sarcoma, also known as chloroma, granulocytic sarcoma, extramedullary Acute Myeloid Leukaemia (AML), myeloblastoma or extramedullary myeloid tumour, is a rare manifestation, characterised by the proliferation of immature myeloid cells, myeloblasts or monoblasts occurring as one or more tumour at an extramedullary site. It is associated with disruption of normal architecture of tissue in which it is found. Monoblastic sarcoma is a rare variant of myeloid sarcoma. Hereby, the authors report a rare case of primary monoblastic sarcoma in a 64-year-old male patient presented with complaint of swelling over right side lower cervical region. He had no history of AML. On physical examination, the swelling was over medial one-third of right clavicle measuring 6×5.8×3 cm. The swelling was excised and sent for histopathological examination, which was diagnosed as monoblastic sarcoma, and confirmed on immunohistochemistry.

A Rare Presentation of ph Chromosome: idic der (22q11) in Blast Crisis Chronic Myeloid Leukemia

The ider(22)t(9;22)(q34;q11) is a rare secondary karyotypic abnormality of Ph chromosome positive chronic myeloid leukemia associated with disease progression, poor clinical outcome and short survival in most of the previously reported cases. The present case of CML with idic der(22)t(9;22)(q34;q11) or idic der Ph with hybrid transcript ratio of BCR-ABL being 97% showed a complex karyotype: 48,XY,+8,t(9;22)(q34;q11)i dic(22)(q11),+idic der(22)t(9;22)(q34;q11). On treatment with Imatinib the initial transcript ratio was reduced to 12.125%, 0.932% but later increased to 93%. The patient developed extra medullary myeloid cell tumor of testis after a year of treatment followed by the detection of T3151 mutation. Cytogenetics provides an evidence for progression of disease at an earlier phase than other markers in this case.

Myeloid neoplasm with eosinophilia associated with isolated extramedullary FIP1L1/PDGFRA rearrangement

Myeloid neoplasms with eosinophilia associated with PDGFRA rearrangement are very responsive to tyrosine kinase inhibitors (TKIs). Herein, we report a case of a 53-year-old man with eosinophilia and a well-differentiated extramedullary myeloid tumor with evidence of FIP1L1/PDGFRA rearrangement by fluorescent in situ hybridization in the extramedullary tissue. His bone marrow evaluation revealed a hypercellular marrow with eosinophilia but without evidence of a FIP1L1/PDGFRA rearrangement. The patient was treated with imatinib at a dose of 100 mg daily and responded with normalization of his peripheral eosinophil count. The case raises the possibility that an extramedullary myeloid tumor may represent a primary site for PDGFRA rearrangement, and highlights the importance of performing cytogenetic testing on extramedullary tissue. Detection of the chromosomal rearrangement is critical for initiation of effective targeted therapy that can improve patient outcomes.

Acute Myelogenous Leukemia Presenting as Acute Pancreatitis: A Case of Primary Pancreatic Extramedullary Acute Myeloid Leukemia

Pancreatitis is a rare manifestation of acute myeloid leukemia (AML) arising during or after the course of the disease. It can be the first and only manifestation of AML. We report a 28-year-old male who presented with epigastric pain. Initial amylase was 1,354 U/L, thus managed as Acute Pancreatitis and improved. Two weeks after, patient returned with left upper quadrant pain and vomiting. Initial hemogram revealed leukocytosis with neutrophilic predominance and lymphopenia; amylase three-folds elevated. Endoscopic retrograde cholangiopancreatography (ERCP) advised. After three days repeat complete blood count (CBC) showed leukocytosis with lymphocytic predominance, presence of myelocytes, metamyelocytes and stabs, and thrombocytopenia; and patient developed yellowish skin discoloration, right upper quadrant pain and anorexia. Abdominal findings revealed tenderness at the epigastric and right upper quadrant areas, with splenomegaly on palpation. Patient was managed as Obstructive Jaundice secondary to Choledocholithiasis by the Surgical Department. He was transferred to Internal Medicine service and managed as Chronic Myelogenous Leukemia in Blast Crisis, Acute Pancreatitis. Peripheral blood smear (PBS) revealed leukemic blast with multiple nucleoli, resembling myeloblast. Induction chemotherapy with Doxorubicin and Cytarabine advised. Computed Tomography (CT) scan of the abdomen revealed an enlarged pancreas with low density areas at the pancreatic head, mass could not be ruled-out. The pancreas has rarely been reported as a site for leukemia. Extramedullary myeloid tumor in AML is usually associated with crisis or leukemic transformation. Clinicians should entertain pancreatic extramedullary acute myeloid leukemia when pancreatic enlargement develops during or after onset of AML.

Extra-adrenal myelolipoma presenting in the spleen: A report of two cases

Myelolipoma is a rare neoplasm composed of mature fat and bone marrow occurring most frequently in the adrenal gland with rare occurrences in extra adrenal locations including lung, liver, retroperitoneum, mediastinum and testes. Splenic myelolipomas are seen most commonly in non-human species including cat and dog. Only rare cases of splenic myelolipoma in humans have been reported previously. We present two cases of myelolipoma in the spleen. The first is a 62year old female presenting with abdominal pain and a splenic mass. The second is a 44year old male presenting with hematuria and a mass in the spleen. Both cases showed trilineage bone marrow elements with mature fat. These cases demonstrate that myelolipoma do rarely occur in human spleen and we highlight the distinction from extramedullary hematopoiesis, mature extramedullary myeloid tumor (myeloid sarcoma), lipoma and well differentiated liposarcoma.

Primary vaginal myeloid sarcoma: a rare case report and review of the literature.

Myeloid sarcoma (chloroma, granulocytic sarcoma, or extramedullary myeloid tumour) is an extramedullary mass forming neoplasm composed of myeloid precursor cells. It is usually associated with myeloproliferative disorders but very rarely may precede the onset of leukemia. Here, we are presenting a rare case of primary vaginal myeloid sarcoma in a geriatric female patient without initial presentation of acute myeloid leukemia (AML). A 68-year-old female patient with ECOG Performance Score of 1 presented with pervaginal bleeding for 20 days. On colposcopic examination, she was found to have mass in the anterior fornix of vagina. A punch biopsy specimen revealed chloromatous infiltration of the vagina. LCA (leukocyte common antigen), MPO (myeloperoxidase), and c-kit were strongly positive on IHC (immunohistochemistry). The patient's routine blood investigations were normal including peripheral smear, lactose dehydrogenase, uric acid, 2D echocardiography, conventional cytogenetics, bone marrow aspiration, and biopsy. The patient was given 4 cycles of decitabine (Decitex, manufactured by Sun Pharmaceutical Industries Limited, India), 20 mg/m2 for 5 days at an interval of 28 days. There was a partial response to decitabine according to RECIST criteria. As decitabine therapy was well tolerated, we are continuing in the same way until disease progression without any complications. The patient is undergoing regular follow-up at our centre.

Primary Granulocytic Sarcoma of Cervix (PGSC): Review and Analysis of 13 Cases

Introduction: Primary granulocytic sarcoma (PGS) is an extramedullary tumor of immature granulocytic cells without prior evidence of leukemia，it often develops to AML by months to years, carrying a poor prognosis. PGS that occur in cervix are rare. The case reports or retrospective case series about it are scarce too. The tumor may involve in any organ and there is no abnormality in bone marrow, so clinicians always confronted with diagnostic and therapeutic challenges. This abstract aims at assisting physicians precisely diagnosing the PGSC as soon as possible and may indicate the timely appropriate therapy which can reduce the risk progressing to AML.Methods: we retrospectively reviewed the database of PubMed and CNKI between January 2003 to May 2014, using the term “Myeloid sarcoma” or “Granulocytic sarcoma” or “Extramedullary Myeloid Cell Tumor” and “cervix”; we also evaluated potentially relevant reports referenced in retrospective case series and a case of PGSC diagnosed in our hospital. The patients’ age, initial and accompanied symptoms, pathological section and Immunohistochemical staining of the tumor, as well as the treatment and prognosis were analyzed.Results: There are 13 cases of the PGSC, 11 of which reported in China and 2 reported abroad. Their age range from 28 to 46 years; These PGSC cases had various presentations in the form of irregular vaginal bleeding (11/13), pain of underbelly (2/13),with other accompanied symptoms such as dyspareunia and increasing of leucorrhea. All the cases made definite diagnosis through pathology and a series of appropriate immunohistochemical panel of antibodies that includes myeloperoxidase, lysozyme,CD68,CD43,CD34,CD20,CD3,CD117,CD45;the tumor cells of all the 13 cases showed positive immunohistochemical reactivity with MPO and at least one of the myelocytic antigens (HLA-DR,CD34,CD117, etc) was expressed. The following antibodies: CD20, CD3 did not react in the neoplastic cells. Several of the cases be mistaken for lymphoma as the two may be indistinguishable on routine hematoxylin and eosin staining; PGS cells, nuclei are slightly smaller with more finely dispersed chromatin, and some cells may show recognizable myeloid differentiation. Most of them received systemic chemotherapy associated with AML, the induce chemotherapy included DA (6/14), IA (2/14), HA (1/14); 2 of the 14 patients received paclitaxel (TAX) combined with chemotherapy;3 patients underwent combination therapy united with operations, radiotherapy and chemotherapy. To prevent the leukemia localization of the central nervous system, the patient in our hospital received intrathecal injection (MTX+Ara-c+Dex), others in the reviewed literature not seen. She has finished her first combined chemotherapy and local radiotherapy of pelvic cavity. PET-CT after the fifth chemotherapy showed complete resolution of the disease site. After 6 months, BM examination showed progression to AML-2a. Leukemia cells were detected quantification of the WT1 and ABL showed 2.49×10^4;and ABL1 7.52×10^4.Leukemia cells were showed negative for AML/ETO1 and Flt-3. Other patients reported in the literature kept a leukemia-free survival state during the follow-up period ranged from 2 months to 6 years.Conclusions: Based on our review of the literature and the case diagnosed in our hospital, the majority of the PGSC patients are presented with vaginal or postcoital bleeding. When the tumor mass is observed in an unusual location without abnormal of hematology, many patients are misdiagnosed or never diagnosed. To make a precise diagnosis, the use of a series of appropriate immunohistochemical panel of antibodies that includes myeloperoxidase, lysozyme, CD68, CD43, CD34, CD20, CD3, however, can successfully identify the vast majority of PGSC. Besides, examinations such as BM aspiration, Cytogenetic, FISH tests and fusion gene were required to make sure whether it is accompanied with AML and monitor the minimal residual disease or indicate a poor risk category. As a systemic disease, combination therapy should united with operations, radiotherapy and chemotherapy, especially the early and intensive chemotherapy can improve prognosis and help to prevent or delay AML, HSCT is also recommended. To prevent the leukemia localization of the central nervous system, intrathecal injection may aid to patients’ longer survival. DisclosuresNo relevant conflicts of interest to declare.

Primary granulocytic sarcoma: a rare presentation of an ovarian mass

Background: Primary granulocytic sarcoma (GS) only rarely presents with an ovarian mass classified as extramedullary myeloid progenitor tumor cells. Few patients with cases of primary GS survive after systemic chemotherapy. Objective: To present a case of primary GS treated with complete course of chemotherapy. The patient is currently in complete remission. Methods: Retrospective review of medical records and comparison with previous case reports. Results: A 41-year-old woman presented with an ovarian mass of 6 months duration. There was no previous history of hematologic disease or presence of leukemic cells in other organs at the time of diagnosis. Immunohistochemistry confirmed the diagnosis. She received complete course of chemotherapy, after which complete remission was achieved. Conclusion: A case of primary granulocytic sarcoma of the ovary was successfully treated with chemotherapy. This disease needs careful diagnosis and appropriate treatment for a good outcome.

Acute Myeloid Leukemia Presenting as Acute Bulbar Palsy

Myeloid Sarcoma1 (also termed as chloroma, granulocytic sarcoma, extra medullary myeloid tumor) is a rare extra medullary tumor composed of immature myeloid cells (myeloblast)2. It is usually associated with leukemia or other myeloproliferative disorder. Myeloid Sarcoma in the central nervous system, around the brain stem is the commonest site of presentation and require high suspicion for diagnosis. We report a forty years male patient with history of dysphagia, dysphonia for last two months. MRI showed chloromas around the brain stem. Laboratory investigations revealed the presence of AML. This is a rare case of Myeloid Sarcoma around the brain stem in a patient of AML. DOI: http://dx.doi.org/10.3329/bjms.v13i4.16056 Bangladesh Journal of Medical Science Vol.13(4) 2014 p.481-483

Extramedullary Myeloid Tumor Involving the Pancreas: A Case Report and Review of the Literature

Extramedullary myeloid tumors (EMMTs) are the tumors of myeloid cells. These tumors may occur in all of the organs of the body, but some localizations are rare. Pancreatic involvement of EMMTs is a rare entity. Here we report a case of EMMT of the pancreas 4 years after allogeneic stem cell transplantation and we review the existing data about EMMTs involving the pancreas.

An unusual cause of thigh swelling: extramedullary myeloid tumor.

Memis Hilmi Atay1, Engin Kelkitli2, Piltan Buyukkaya3, Kubilay Ekiz4, Levent yildiz5, Mehmet Turgut3 1Van Training and Research Hospital, Department of Internal Medicine, Division of Hematology, Van, Turkey 2Erzurum Training and Research Hospital, Department of Internal Medicine, Division of Hematology, Erzurum, Turkey 3Ondokuz Mayis University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Samsun, Turkey 4Ondokuz Mayis University Faculty of Medicine, Department of Internal Medicine, Samsun, Turkey 5Ondokuz Mayis University Faculty of Medicine, Department of Pathology, Samsun, Turkey

Clinical report. Primary granulocytic sarcoma: a rare presentation of an ovarian mass

Abstract Background: Primary granulocytic sarcoma (GS) only rarely presents with an ovarian mass classified as extramedullary myeloid progenitor tumor cells. Few patients with cases of primary GS survive after systemic chemotherapy. Objective: To present a case of primary GS treated with complete course of chemotherapy. The patient is currently in complete remission. Methods: Retrospective review of medical records and comparison with previous case reports. Results: A 41-year-old woman presented with an ovarian mass of 6 months duration. There was no previous history of hematologic disease or presence of leukemic cells in other organs at the time of diagnosis. Immunohistochemistry confirmed the diagnosis. She received complete course of chemotherapy, after which complete remission was achieved. Conclusion: A case of primary granulocytic sarcoma of the ovary was successfully treated with chemotherapy. This disease needs careful diagnosis and appropriate treatment for a good outcome.

Granulocytic sarcoma of the breast: a case study in LAO PDR

Granulocytic sarcoma (GS) also called, myeloid sarcoma, chloroma, myeloblastoma or extra medullary myeloid tumor is an extra medullary hematopoietic neoplasm composed of immature myeloid cell (myeloblasts). GS may occur in leukemia, myeloproliferative disorder. GS can occur in any site of the body; common sites of involvement include bone, CNS, soft tissue, lymph nodes and skin, but breast is very rare. GS of breast have been reported both before and after hematological diagnosis. Primary, isolate or non-leukemic GS of breast was described when bone marrow biopsy to confirm as no other hematologic malignancy. Depending on review of all Medline cases reported, only 24 cases of primary were commonly in unilateral site, and only 4 cases were bilateral site. The incidence of GS of breast is 2/1,000,000 in adults and ages ranged from 16–72 years (mean 31 years). The histology was composed of homomorphous of eosinophil cytoplasm of immature cell with single or multiple nucleoli. The malignant cells were positive for myeloperoxidase (MPO), CD43, CD34, TDT, CD 117, CD68 and negative for CD20, CD30, CD3, CD56, CD79 and ULH1.The differential diagnosis included lymphoma and poorly differentiated carcinoma. The prognosis was very poor. We report a case of granulocytic sarcoma (GS) in the left breast of a 33-year-old woman who presented with breast mass, which was diagnosed as diffuse lymphoma, large cell type on H&E histopathology without any special straining, immunohistochemical and very limited of patient information such as echography, CBC, FNA or biopsy of breast or bone marrow tissue. The final diagnosis was made by additional immunohistochemical study.

Acute Myeloid Leukemia Concurrent with Spinal Epidural Extramedullary Myeloid Sarcoma Accompanied by a High CD25 Expression and the <i>FLT3-ITD</i> Mutation

Myeloid sarcoma (MS) is an extramedullary myeloid tumor that sometimes presents with antedating systemic leukemia, leading physicians to the misdiagnosis of lymphoma. CD25 is expressed in 13% of patients with acute myeloid leukemia (AML), and its expression is associated with FLT3-ITD mutations, an elevated serum soluble interleukin 2 receptor (sIL-2R) level and a lower survival rate. However, there are no reports concerning the relationship between MS and the CD25 expression. We herein report a case of AML accompanied by thoracic epidural MS with a high CD25 expression, the FLT3-ITD mutation and an extremely elevated serum sIL-2R level in a 59-year-old man who presented with paraplegia.

Management of isolated recurrence of extramedullary myeloid tumor at a single site

Extramedullary myeloid tumors (EMMT) can precede, occur with or follow AML. Rarely, they can present as isolated relapses. We present a 9-year-old child with t (8, 21) positive AML who was treated with induction regimen and achieved remission. While on high-dose cytarabine consolidation, he had isolated relapse in a single cervical lymph node with uninvolved marrow. He was treated with salvage chemotherapy alone. There are no clear guidelines for treatment of isolated extramedullary relapse. Chemotherapy, radiotherapy followed by stem cell transplant is the usual option. Our patient is unique for the unusual site of relapse and prolonged remission with cladribine-based chemotherapy, mitoxantrone-based consolidation, and oral maintenance therapy.

Extramedullary tumors and leukemia

To describe neurologic presentations and radiologic findings of extramedullary myeloid tumor (EMT). This is a retrospective case series of patients with neurologic presentations of EMT from January 1, 1981, until May 30, 2011. Clinical data abstracted included demographics, presentation, bone marrow involvement, history of hematologic malignancy, complete blood count at presentation, EMT location, imaging findings, treatments, and outcomes. Of 21 patients, EMT was the initial presentation of underlying hematologic disorder in 12 (57%). Six patients (29%) presented with primary EMT (no bone marrow involvement at presentation). The most common location was the thoracic spine (n = 9), usually manifesting as an epidural mass with vertebral body involvement. The initial diagnosis was incorrect in most (n = 17 [81%]). Treatments included radiation (n = 17 [ 81%]), chemotherapy (n = 14 [67%]), and surgery (n = 6 [29%]). Fifteen patients (71%) died. Estimated Kaplan-Meier median survival from diagnosis for 20 patients with adequate follow-up was 8 months. Of the 6 patients with primary EMT (no known systemic leukemia at diagnosis), 5 died at a median of 24 months (range 8-36 months) and 1 is still alive at 1 year. Of the 6 patients whose leukemia was diagnosed upon presenting with EMT, 3 are still alive. EMT affecting the nervous system can present in patients without a known hematologic disorder and is often not recognized. Thoracic paraspinal masses are the most common presentation. The prognosis for patients with neurologic presentation of EMT is generally poor, but longer survival is possible in patients presenting with isolated EMT or leukemia first diagnosed at the time of EMT presentation.

Multiple Jejunal Myeloid Sarcomas Presenting with Intestinal Obstruction in a Non-leukemic Patient: A Case Report with Ultrastructural Observations

Myeloid sarcoma is a rare extramedullary myeloid tumor, which is frequently misdiagnosed when no evidence of leukemia is initially observed. Here, we report on a peculiar case of a 49-year-old man afflicted with multiple masses in the jejunum, the superior mesentery, and the serosa of the transverse colon, without leukemic manifestation. The tumor was composed of undifferentiated small round cells containing eosinophilic cytoplasm, which were negative for myeloperoxidase, nonspecific esterase, lysozyme, terminal deoxynucleotidyl transferase, leukocyte common antigen, CD3, CD4, CD15, CD20, CD30, CD43, CD56, CD68/PG-M1, CD79a, human melanoma black-45, c-kit, and CD34 with positivity only for CD68/KP1, CD99, and vimentin. Under electron microscopy, those cells had abundant membrane-bound cytoplasmic granules that measured 200 to 300 nm in diameter, which were consistent with granulocytic azurophilic granules. The tumor was finally diagnosed as a myeloid sarcoma. The presence of non-leukemic myeloid sarcomas showing immunonegativity for conventional myeloid-leukemic markers necessitated a diagnosis by ultrastructural observation.