Image Description: Peripheral blood smear showing an increas | Figure 1
JIMHICR
Journal of Investigative Medicine
Image Description: Peripheral blood smear showing an increased number of lymphocytes. Both small, mature lymphocytes and larger cells with less condensed nuclear material are seen. Numerous acanthocytes are present. This could be a consequence of congestive splenomegaly, marrow infiltration by tumor (myelophthisis), or both.
Case Description: B-cell prolymphocytic leukemia (B-PLL) is a rare leukemia characterized by rapidly increasing leukocytosis with splenomegaly and lymphadenopathy. Treatment strategies are largely based on studies of chronic lymphocytic leukemia (CLL). Antibodies against the cell surface protein CD20 are considered to be first-line therapy. A 76-year-old male with known CLL presented 2 weeks after starting chemoimmunotherapy for newly refractory CLL after failing ibrutinib therapy. White blood cell count was elevated at 226.7 × 103/µL. Fluorescent in situ hybridization analysis of a bone marrow specimen showed new development of complex cytogenetics. Flow cytometry revealed B cells appearing slightly dimmer on CD45 and brighter on CD20 compared with typical B-CLL suggestive of less mature lymphocyte forms. The patient was diagnosed with B-PLL and started on obinutuzumab and venetoclax with rapid normalization of white blood cells. This case recapitulates the challenges in diagnosing and treating B-PLL. Ibrutinib resistance is a growing area of study with several proposed mechanisms of acquired resistance. The pathogenesis of B-PLL is not completely understood, although mutations in MYC are presumed to play a role.
Chronic lymphocytic leukemia (CLL) is an indolent malignancy characterized by the accumulation of dysfunctional B-cell lymphocytes. Complications such as hemophagocytic lymphohistiocytosis (HLH) can arise, particularly during disease progression. HLH has been increasingly reported as a complication of CLL, often triggered by factors such as superimposed infections, chemotherapy, Richter transformation, or disease progression. This case explores HLH as an initial presentation of undiagnosed CLL without any identifiable trigger. We present the case of a 65-year-old woman who presented with a high-grade fever, sore throat, and pancytopenia. Despite broad-spectrum antibiotic treatment, her condition deteriorated. Investigations revealed elevated ferritin levels, low natural killer cell activity, and other findings consistent with HLH. Flow cytometry and bone marrow biopsy ultimately confirmed the diagnosis of CLL. HLH is characterized by the hyperactivation of immune cells and is known to be triggered by a variety of factors, including infections and malignancies. In this case, the absence of identifiable triggers raises important questions about the underlying pathophysiology linking HLH with CLL. While previous reports have highlighted HLH as a complication of CLL, typically secondary to infection or treatment, this case is particularly noteworthy due to the unexplained onset of HLH in the absence of such triggers. This case underscores the need for heightened awareness of HLH as a potential manifestation of underlying malignancy, especially in non-septic patients presenting with unexplained fever and pancytopenia. In addition, the simultaneous presentation of normal pressure hydrocephalus emphasizes the complex interplay of inflammatory processes in CLL. Further research is needed to explore the relationship between inflammation and the pathogenesis of CLL.
This is a case of a 17-year-old female presenting with fever, body malaise, and a 1-year history of hematoma on her trunk and extremities. Initial CBC and peripheral smear pointed to pancytopenia so the attending considered aplastic anemia, thus bone marrow study was requested. On examination, the bone marrow aspirates were hypercellular with complete sequential maturation and several myeloblasts, promyelocytes, and myelocytes exhibited auer rods. Diagnosis: ACUTE MYELOGENOUS LEUKEMIA WITH MATURATION (FAB - AML M2).
A 52-year-old man presented with a 20-minute history of chest pain radiating to the left shoulder and neck, associated with dyspnea and diaphoresis. He had a history of hypertension, long-term smoking, and a sedentary lifestyle. On physical examination, bibasilar crackles were noted on lung auscultation. An electrocardiogram (ECG) was performed in the primary care setting, and the patient was promptly referred to the emergency department.
A 28-year-old woman presented with a 3-day history of intense pruritus over the lower back. She reported no recent travel or exposure to new environments. She cares for a small kennel with approximately eight dogs rescued from the streets. Physical examination revealed multiple small vesicles, some clustered and others scattered, predominantly involving the lumbar region and the left gluteal area.
A 4-month-old male infant presented with skin lesions localized to the chin for the past 3 days. Physical examination revealed multiple small pustules with surrounding inflammatory signs on the chin, along with a few scattered papules on the chest.
Patient in late teens presenting after a collapse, no chest pain, no previous cardiac history, no history of sudden death in family, are there any features in this ECG that would warrant further work-up or is this just a pediatric ECG?