Amr Hanbali

Severe lactic acidosis in a patient with metastatic prostate cancer

Lactic acidosis (LA) due to malignancy was first reported in patients with acute leukemia. Since then, several malignancies have been reported to be associated with LA. The pathophysiology of cancer-related LA is multifactorial and still poorly understood. In general, chemotherapy is the only effective mean of correcting malignancy-related LA by cytoreduction of the tumor cells while at the same time decreasing malignant liver involvement leading to improved clearance of lactic acid. LA is rare in patients with malignancies and is usually associated with high mortality because of advanced disease process and high tumor burden. Increased awareness of this complication in certain malignancies is important because early initiation of chemotherapy may decrease LA and perhaps prolong survival. To our knowledge, this is the first case of otherwise unexplained severe LA in a patient with chemotherapy-refractory metastatic prostate cancer.

The Evolution of Prognostic Factors in Multiple Myeloma

Multiple myeloma (MM) is a heterogeneous hematologic malignancy involving the proliferation of plasma cells derived by different genetic events contributing to the development, progression, and prognosis of this disease. Despite improvement in treatment strategies of MM over the last decade, the disease remains incurable. All efforts are currently focused on understanding the prognostic markers of the disease hoping to incorporate the new therapeutic modalities to convert the disease into curable one. We present this comprehensive review to summarize the current standard prognostic markers used in MM along with novel techniques that are still in development and highlight their implications in current clinical practice.

Epidermal Growth Factor Receptor-Induced Hirsutism and Trichomegaly

© 2011 Mayo Foundation for Medical Education and Research A 48-year-old nonsmoking Asian woman presented with cough 3 years previously. Computed tomography of the thorax (panel A) showed possible multisegmental pneumonia in the right lung, and antibiotics were initiated. Because there was no clinical or radiologic improvement, bronchoscopy was performed and confirmed welldifferentiated adenocarcinoma of the lung, which was deemed to be unresectable. The epidermal growth factor receptor mutation status of the tumor was negative. The patient received 6 cycles of carboplatin, paclitaxel, and bevacizumab; the disease stabilized, and subsequently maintenance therapy with the tyrosine kinase inhibitor erlotinib was initiated due to her phenotype and despite her negative epidermal growth factor receptor mutation status. Interval computed tomography of the thorax (panel B) showed clear response to erlotinib therapy; thus, the patient continued this medication for almost 2 years even though she developed hirsutism (panel C), mild rash, and trichomegaly (panels D and E) that required regular trimming of her eyelashes. Results of hormonal studies were within normal levels. Ultimately, the patient’s lung cancer progressed, and erlotinib use was discontinued. Single-agent pemetrexed was initiated, and the hirsutism, skin rash, and trichomegaly resolved during the subsequent 6 months.

Sequential occurrence of thrombotic thrombocytopenic purpura, essential thrombocythemia, and idiopathic thrombocytopenic purpura in a 42-year-old African-American woman: a case report and review of the literature

IntroductionThrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura are two well recognized syndromes that are characterized by low platelet counts. In contrast, essential thrombocythemia is a myeloproliferative disease characterized by abnormally high platelet numbers.The coexistence of thrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura in a single patient has been reported in the literature on a few occasions. However, having essential thrombocythemia complicating the picture has never been reported before.Case presentationWe present a case where thrombotic thrombocytopenic purpura, essential thrombocythemia, and idiopathic thrombocytopenic purpura were diagnosed in a 42-year-old African-American woman in the space of a few years; we are reporting this case with the aim of drawing attention to this undocumented occurrence, which remains under investigation.ConclusionsAs the three conditions have different natural histories and require different treatment modalities, it is important to recognize that these diseases may be seen sequentially. This case emphasizes the importance of reviewing peripheral blood smears for evaluation of thrombocytopenia and bone marrow aspirations for diagnosis of thrombocythemia in order to reach an accurate diagnosis and tailor therapy accordingly. Moreover, this case demonstrates the variability and complexity of platelet disorders. This occurrence of three different types of platelet disorders in one patient remains a pure observation on our part; regardless, this does raise the possibility of a common underlying, as yet undiscovered, pathophysiology that could explain the phenomenon.

Plasma cell leukemia mimicking hairy cell leukemia

A42-year-old female presented with fever and shortness of breath, and was diagnosed with community acquired pneumonia. On presentation she was found to have a white cell count of 13 · 10/L, hemoglobin of 7.1 g/dL and platelets of 51 · 10/L. Peripheral blood smear showed left shift and many cells with hairy projections (56%) (See Fig. 1) that were positive for CD38 and CD138 by peripheral blood flow cytometry. Other laboratory findings included B-2 microglobulin of 15.3 mg/L with normal creatinine and calcium levels. Serum protein electrophoresis evaluation revealed monoclonal gammopathy (IgG-lambda) of 45.87 g/L. Urine protein electrophoresis showed the presence of Ig-G lambda as well as free-lambda light chain monoclonal bands. Bone marrow biopsy showed increased cellularity (95%) with increased number of plasma cells (45%). Flow cytometry showed 44% of the cells to have clonal plasma cell phenotype (CD38+ve, CD138+ve, CD20 ve, CD22 ve, and cytoplasmic lambda light chin restriction). A diagnosis of plasma cell leukemia was made and treatment was started with bortezomib, cyclophosphamide, and dexamethasone. The patient achieved partial remission. Treatment was followed by autologous bone marrow transplantation and then two cycles of lenalidomide and dexamethasone consolidation. The patient subsequently achieved complete remission. She has been on maintenance lenalidomide over the past six months and continues to be in complete remission.

Progression of double‐hit lymphoma in the midst of R‐hyper CVAD

A 50-year-old female with no medical history presented with B-symptoms for 2 weeks including generalized weakness, fevers, fatigue, and night sweats. Physical exam revealed diffuse cervical and axillary lymphadenopathy with splenomegaly. Initial laboratories showed Coombs-negative hemolytic anemia with agglutination of red blood cells and moderate thrombocytopenia. Some tear drop cells were also seen pointing toward the presence of bone marrow infiltration. Computed tomography (CT) of the body showed a mediastinal mass with widespread lymphadenopathy and splenomegaly. Image 1. Panel A: Right axillary lymph node excisional biopsy showing BCL-2 positivity. Panel B: Right axillary lymph node excisional biopsy showing CD10 positivity. Panel C: Right axillary lymph node excisional biopsy showing follicular low-grade lymphoma. Panel D: Right axillary lymph node excisional biopsy showing low MIB1. Panel E: Mediastinal mass biopsy showing DLBCL with blastoid features. Panel F: Mediastinal mass biopsy showing high MIB1. Panel G: Baseline CT showing a mediastinal mass. Panel H: The mediastinal mass rapidly responded to high dose chemotherapy achieving radiographic complete response after three cycles. Panel I: CT showing recurrence of the mediastinal mass encasing the major thoracic organs.

Fulminant Malignant Hepatic Failure

We present a patient that developed abdominal pain, jaundice and confusion leading to a diagnosis of metastatic disease to the liver. Our case is a reminder of the possibility of malignancy in the differential diagnosis of fulminant liver failure. The liver is a common destination for metastatic malignant spread nevertheless, metastatic fulminant liver failure is a rare occurrence and when it develops is usually hematological in nature. Prognosis has remained poor over the last decades as most patients have a fatal outcome within days after their admission to the hospital for work-up of hyperbilirubinemia. Malignant hepatic infiltration should be considered in the differential diagnosis of acute progressive liver failure.

Can rituximab replace splenectomy in immune thrombocytopenic purpura

AimImmune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by premature platelet destruction. Standard practice is to initiate treatment with corticosteroids, followed by splenectomy. Most published literature for responses from rituximab is in patients with chronic refractory ITP, who have failed multiple prior treatments, including splenectomy. We therefore decided to analyze our patient population with ITP who had been treated with rituximab, mainly as a second line treatment regimen prior to splenectomy.MethodsWe performed a retrospective chart review of patients with a diagnosis of ITP who had been treated with rituximab between January 2001 and December 2006 at our institution.Results18/29 patients (62%) had a CR, 2/29 (7%) patients had a PR, representing an overall response rate of 69%. The average time to response was 5 weeks and all patients have maintained their response for more than 12 months after treatment with rituximab.ConclusionOur study shows higher CR, comparable overall response rates, but with a longer duration of response when compared to the published literature.

A case of T-cell lymphoproliferative disorder associated with hypereosinophilia with excellent response to mycophenolate mofetil.

Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by a persistent elevation of blood eosinophil count ⩾1.5×109/L and clinical manifestations attributable to eosinophilia or tissue hypereosinophilia. Lymphocytic variant of HES (HES-L) is a known subtype according to World Health Organization classification. It is well documented in the literature that patients with HES-L are predisposed to develop T-cell lymphoma. We report a case of T-cell lymphoproliferation associated with hypereosinophilia, which has been successfully treated with mycophenolate mofetil, with resolution of skin lesions and normalization of eosinophil count and immunoglobulin E level. We believe this is a clinically relevant case since this is a rare disease with little known knowledge on its best treatment modality.

Philadelphia-Like acute lymphoblastic leukemia: diagnostic dilemma and management perspectives

Acute lymphoblastic leukemia (ALL) is an aggressive hematologic malignancy characterized by suboptimal outcomes in the adult age group. Recently, a new subtype called Philadelphia (Ph)-like ALL has been described. This subgroup is characterized by high cytokine receptor and tyrosine kinase signaling expression, resulting in kinase activation through stimulation of two main pathways, the ABL and JAK/STAT pathways. The diagnostic method or approach for Ph-like ALL is still not standardized and efforts are ongoing to identify an easy and applicable diagnostic method. Accurate and standard testing approaches are much needed and this will facilitate better understanding of this subgroup, including better estimation of the prevalence and incidence in different age groups and the clinical outcomes of such new entity. Here, we review the currently available diagnostic tools, activated pathways, and different therapeutic approaches used to target this subgroup.