An Uncommon Complication of Chronic Myelomonocytic Leukemia: Acute Monocytic Transformation and Leukostasis

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Background: Chronic Myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy and remains the most common overlapping myelodysplastic/ myeloproliferative neoplasm. Complications of CMML to acute monocytic leukemia transformation may include leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation. We report a case that illustrates the disease characteristics and complications of CMML.

Case Report: A 76-year-old man, who was diagnosed 3 months prior to presentation with CMML, presented to the emergency department with dyspnea, altered mental status and right upper quadrant abdominal pain. His white count on admission was 314.5 × 109/L, which included 50 × 109/L blasts. His liver enzymes were elevated, and initial lactic acid was 12.5. No source of infection was found on imaging with Computed Tomography. Flow cytometry of peripheral blood revealed Acute Monocytic Leukemia. The diagnosis was consistent with CMML transformation to acute monocytic leukemia with hyperleukocytosis and leukostasis. He was started on cytoreduction therapy with hydroxyurea, leukapheresis, and decitabine chemotherapy. His symptoms improved with normalization of his white cell counts and he was discharged to concurrent hospice.

Discussion: Blast transformation (BT) to acute monocytic leukemia occurs in about 14% of patients diagnosed with CMML. 10-20% of newly diagnosed acute myeloid leukemia patients develop hyperleukocytosis (white cell counts greater than 100 x 109/L), a laboratory abnormality which may manifest clinically as leukostasis. Leukostasis, diagnosed empirically in a patient with BT who presents with respiratory and neurological symptoms, has 1-week mortality of 20%-40% if left untreated. Treatment of leukostasis includes leukapheresis, hydroxyurea, and induction chemotherapy. Recent studies have shown that leukapheresis reduces four-week mortality but does not affect long term mortality rate. More research is needed in understanding the pathophysiology of leukostasis, thus paving the way for novel therapeutic agents.

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Correspondence: Hussain Ismail Rangoonwala (hussainrangoonwala@creighton.edu)

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Correspondence: Hussain Ismail Rangoonwala (hussainrangoonwala@creighton.edu)

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Correspondence: Hussain Ismail Rangoonwala (hussainrangoonwala@creighton.edu)

Background: Chronic Myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy and remains the most common overlapping myelodysplastic/ myeloproliferative neoplasm. Complications of CMML to acute monocytic leukemia transformation may include leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation. We report a case that illustrates the disease characteristics and complications of CMML.

Case Report: A 76-year-old man, who was diagnosed 3 months prior to presentation with CMML, presented to the emergency department with dyspnea, altered mental status and right upper quadrant abdominal pain. His white count on admission was 314.5 × 109/L, which included 50 × 109/L blasts. His liver enzymes were elevated, and initial lactic acid was 12.5. No source of infection was found on imaging with Computed Tomography. Flow cytometry of peripheral blood revealed Acute Monocytic Leukemia. The diagnosis was consistent with CMML transformation to acute monocytic leukemia with hyperleukocytosis and leukostasis. He was started on cytoreduction therapy with hydroxyurea, leukapheresis, and decitabine chemotherapy. His symptoms improved with normalization of his white cell counts and he was discharged to concurrent hospice.

Discussion: Blast transformation (BT) to acute monocytic leukemia occurs in about 14% of patients diagnosed with CMML. 10-20% of newly diagnosed acute myeloid leukemia patients develop hyperleukocytosis (white cell counts greater than 100 x 109/L), a laboratory abnormality which may manifest clinically as leukostasis. Leukostasis, diagnosed empirically in a patient with BT who presents with respiratory and neurological symptoms, has 1-week mortality of 20%-40% if left untreated. Treatment of leukostasis includes leukapheresis, hydroxyurea, and induction chemotherapy. Recent studies have shown that leukapheresis reduces four-week mortality but does not affect long term mortality rate. More research is needed in understanding the pathophysiology of leukostasis, thus paving the way for novel therapeutic agents.

Background: Chronic Myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy and remains the most common overlapping myelodysplastic/ myeloproliferative neoplasm. Complications of CMML to acute monocytic leukemia transformation may include leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation. We report a case that illustrates the disease characteristics and complications of CMML.

Case Report: A 76-year-old man, who was diagnosed 3 months prior to presentation with CMML, presented to the emergency department with dyspnea, altered mental status and right upper quadrant abdominal pain. His white count on admission was 314.5 × 109/L, which included 50 × 109/L blasts. His liver enzymes were elevated, and initial lactic acid was 12.5. No source of infection was found on imaging with Computed Tomography. Flow cytometry of peripheral blood revealed Acute Monocytic Leukemia. The diagnosis was consistent with CMML transformation to acute monocytic leukemia with hyperleukocytosis and leukostasis. He was started on cytoreduction therapy with hydroxyurea, leukapheresis, and decitabine chemotherapy. His symptoms improved with normalization of his white cell counts and he was discharged to concurrent hospice.

Discussion: Blast transformation (BT) to acute monocytic leukemia occurs in about 14% of patients diagnosed with CMML. 10-20% of newly diagnosed acute myeloid leukemia patients develop hyperleukocytosis (white cell counts greater than 100 x 109/L), a laboratory abnormality which may manifest clinically as leukostasis. Leukostasis, diagnosed empirically in a patient with BT who presents with respiratory and neurological symptoms, has 1-week mortality of 20%-40% if left untreated. Treatment of leukostasis includes leukapheresis, hydroxyurea, and induction chemotherapy. Recent studies have shown that leukapheresis reduces four-week mortality but does not affect long term mortality rate. More research is needed in understanding the pathophysiology of leukostasis, thus paving the way for novel therapeutic agents.

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Docetaxel-Induced Stevens-Johnson Syndrome

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Abstract 37: 2016 AVAHO Meeting

Abstract: Docetaxel is a commonly used chemotherapeutic agent used in a variety of cancer treatment plans. We present a case of apparent docetaxel-induced Stevens-Johnson syndrome (SJS) in a patient recently treated with docetaxel for metastatic prostate cancer. This medication is not classically associated with the development of SJS.

A 63-year-old gentleman with a past medical history of hypertension, hyperlipidemia, and metastatic prostate adenocarcinoma to retroperitoneal lymph nodes, lung, and bone presented to the emergency room with a one-week history of a rash affecting his hands, feet, back, and chest. It developed into blisters that later ruptured. it was especially painful in the hands and feet. He also reported red eyes and difficulty eating for a week.

Vital Signs: Temp 36.4°C, 83/min, R.R 12/min, BP 121/60 mm Hg. Physical examination of the patient revealed a severe rash covering less than 30% of the body, oral ulcers, and conjunctival redness.

The patient’s cancer treatment regimen included active hormonal therapy with leuprolide and active chemotherapy with docetaxel. He had received 2 cycles of docetaxel therapy (75 mg/m2), with the last dose of docetaxel received 2 weeks prior to presentation.

Treatment of the patient included intravenous fluid replacement, prednisone, piperacillin/tazobactam, ondansetron, and morphine. The skin lesions were kept clean with regular dressing changes.

Because our searches for an established association between SJS and docetaxel were in vain, we elected to obtain a punch biopsy of the lesions to establish pathological evidence of our diagnosis. The punch biopsies were obtained from the edges of lesions on the left forearm and left medial foot. Light microscopy confirmed the diagnosis of SJS (clinical pics, pathology slides are available).

Discussion: Docetaxel is a widely used chemotherapeutic agent in the treatment of breast, lung, prostate and other cancers. The classically known side effects of docetaxel therapy include alopecia, pancytopenia, hepatotoxicity, nausea, vomiting, and diarrhea. A number of popular clinical pharmacology resources do not include Stevens-Johnson syndrome (SJS) as a known complication of docetaxel chemotherapy. However, the current case and the cases written by a handful of other clinicians may provide clinical evidence that docetaxel therapy is associated with the development of this potentially life-threatening dermatologic condition.

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Abstract 37: 2016 AVAHO Meeting
Abstract 37: 2016 AVAHO Meeting

Abstract: Docetaxel is a commonly used chemotherapeutic agent used in a variety of cancer treatment plans. We present a case of apparent docetaxel-induced Stevens-Johnson syndrome (SJS) in a patient recently treated with docetaxel for metastatic prostate cancer. This medication is not classically associated with the development of SJS.

A 63-year-old gentleman with a past medical history of hypertension, hyperlipidemia, and metastatic prostate adenocarcinoma to retroperitoneal lymph nodes, lung, and bone presented to the emergency room with a one-week history of a rash affecting his hands, feet, back, and chest. It developed into blisters that later ruptured. it was especially painful in the hands and feet. He also reported red eyes and difficulty eating for a week.

Vital Signs: Temp 36.4°C, 83/min, R.R 12/min, BP 121/60 mm Hg. Physical examination of the patient revealed a severe rash covering less than 30% of the body, oral ulcers, and conjunctival redness.

The patient’s cancer treatment regimen included active hormonal therapy with leuprolide and active chemotherapy with docetaxel. He had received 2 cycles of docetaxel therapy (75 mg/m2), with the last dose of docetaxel received 2 weeks prior to presentation.

Treatment of the patient included intravenous fluid replacement, prednisone, piperacillin/tazobactam, ondansetron, and morphine. The skin lesions were kept clean with regular dressing changes.

Because our searches for an established association between SJS and docetaxel were in vain, we elected to obtain a punch biopsy of the lesions to establish pathological evidence of our diagnosis. The punch biopsies were obtained from the edges of lesions on the left forearm and left medial foot. Light microscopy confirmed the diagnosis of SJS (clinical pics, pathology slides are available).

Discussion: Docetaxel is a widely used chemotherapeutic agent in the treatment of breast, lung, prostate and other cancers. The classically known side effects of docetaxel therapy include alopecia, pancytopenia, hepatotoxicity, nausea, vomiting, and diarrhea. A number of popular clinical pharmacology resources do not include Stevens-Johnson syndrome (SJS) as a known complication of docetaxel chemotherapy. However, the current case and the cases written by a handful of other clinicians may provide clinical evidence that docetaxel therapy is associated with the development of this potentially life-threatening dermatologic condition.

Abstract: Docetaxel is a commonly used chemotherapeutic agent used in a variety of cancer treatment plans. We present a case of apparent docetaxel-induced Stevens-Johnson syndrome (SJS) in a patient recently treated with docetaxel for metastatic prostate cancer. This medication is not classically associated with the development of SJS.

A 63-year-old gentleman with a past medical history of hypertension, hyperlipidemia, and metastatic prostate adenocarcinoma to retroperitoneal lymph nodes, lung, and bone presented to the emergency room with a one-week history of a rash affecting his hands, feet, back, and chest. It developed into blisters that later ruptured. it was especially painful in the hands and feet. He also reported red eyes and difficulty eating for a week.

Vital Signs: Temp 36.4°C, 83/min, R.R 12/min, BP 121/60 mm Hg. Physical examination of the patient revealed a severe rash covering less than 30% of the body, oral ulcers, and conjunctival redness.

The patient’s cancer treatment regimen included active hormonal therapy with leuprolide and active chemotherapy with docetaxel. He had received 2 cycles of docetaxel therapy (75 mg/m2), with the last dose of docetaxel received 2 weeks prior to presentation.

Treatment of the patient included intravenous fluid replacement, prednisone, piperacillin/tazobactam, ondansetron, and morphine. The skin lesions were kept clean with regular dressing changes.

Because our searches for an established association between SJS and docetaxel were in vain, we elected to obtain a punch biopsy of the lesions to establish pathological evidence of our diagnosis. The punch biopsies were obtained from the edges of lesions on the left forearm and left medial foot. Light microscopy confirmed the diagnosis of SJS (clinical pics, pathology slides are available).

Discussion: Docetaxel is a widely used chemotherapeutic agent in the treatment of breast, lung, prostate and other cancers. The classically known side effects of docetaxel therapy include alopecia, pancytopenia, hepatotoxicity, nausea, vomiting, and diarrhea. A number of popular clinical pharmacology resources do not include Stevens-Johnson syndrome (SJS) as a known complication of docetaxel chemotherapy. However, the current case and the cases written by a handful of other clinicians may provide clinical evidence that docetaxel therapy is associated with the development of this potentially life-threatening dermatologic condition.

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Presentation of Primary Ocular Melanoma in an Adult Male

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Abstract 20: 2016 AVAHO Meeting

Ocular melanoma is the most common primary intraocular malignancy and can often be fatal. It is relatively uncommon and presents in about 5.1 cases per million population per year. Oftentimes, the patient is asymptomatic at diagnosis and the presentation is highly variable. We present a case of ocular melanoma.

A 68-year-old man with a history of hypertension, osteoarthritis, and coronary artery disease came in after having worsening pain in multiple joints. Review of systems revealed worsening blurry vision and eye floaters. He denied eye pain or other associated complaints. He had no past history of any ocular pigmented lesions or history of skin cancer. Ophthalmology evaluation a few years earlier did not identify any abnormalities. Approximately 10 years prior to presentation, he did have LASIK surgery on both eyes. Subsequent ophthalmological evaluation showed an iris mass, elevated pressure, intra-retinal hemorrhages, and evidence of involvement in the choroid and conjunctivae. This was highly suspicious for iris melanoma of the right eye. He was started on intraocular pressure lowering medications and further workup was initiated. Biopsy confirmed the diagnoses of choroidal melanoma with an iris mass measuring 1 mm radially by 4 mm circumferentially. The mass extended posteriorly and involved well over half his iridocorneal angle resulting in very high intraocular pressure. A metastatic workup was done and was negative at the time. He underwent successful enucleation surgery with prostheses placement. Patient did well until about 1.5 years later when he was found to have multiple liver lesions suggestive of metastasis. This is currently being further evaluated.

No current guidelines exist for the screening of primary ocular melanoma as well as for screening for metastasis in those already diagnosed. Unfortunately, up to 50% of patients with ocular melanoma develop metastases. This case opens the discussion of needing current guidelines for screening and better surveillance in ocular melanomas. It highlights the importance of looking into screening using genomics and also developing targeted therapies, as well as focusing on immunotherapies for these cases.

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Abstract 20: 2016 AVAHO Meeting
Abstract 20: 2016 AVAHO Meeting

Ocular melanoma is the most common primary intraocular malignancy and can often be fatal. It is relatively uncommon and presents in about 5.1 cases per million population per year. Oftentimes, the patient is asymptomatic at diagnosis and the presentation is highly variable. We present a case of ocular melanoma.

A 68-year-old man with a history of hypertension, osteoarthritis, and coronary artery disease came in after having worsening pain in multiple joints. Review of systems revealed worsening blurry vision and eye floaters. He denied eye pain or other associated complaints. He had no past history of any ocular pigmented lesions or history of skin cancer. Ophthalmology evaluation a few years earlier did not identify any abnormalities. Approximately 10 years prior to presentation, he did have LASIK surgery on both eyes. Subsequent ophthalmological evaluation showed an iris mass, elevated pressure, intra-retinal hemorrhages, and evidence of involvement in the choroid and conjunctivae. This was highly suspicious for iris melanoma of the right eye. He was started on intraocular pressure lowering medications and further workup was initiated. Biopsy confirmed the diagnoses of choroidal melanoma with an iris mass measuring 1 mm radially by 4 mm circumferentially. The mass extended posteriorly and involved well over half his iridocorneal angle resulting in very high intraocular pressure. A metastatic workup was done and was negative at the time. He underwent successful enucleation surgery with prostheses placement. Patient did well until about 1.5 years later when he was found to have multiple liver lesions suggestive of metastasis. This is currently being further evaluated.

No current guidelines exist for the screening of primary ocular melanoma as well as for screening for metastasis in those already diagnosed. Unfortunately, up to 50% of patients with ocular melanoma develop metastases. This case opens the discussion of needing current guidelines for screening and better surveillance in ocular melanomas. It highlights the importance of looking into screening using genomics and also developing targeted therapies, as well as focusing on immunotherapies for these cases.

Ocular melanoma is the most common primary intraocular malignancy and can often be fatal. It is relatively uncommon and presents in about 5.1 cases per million population per year. Oftentimes, the patient is asymptomatic at diagnosis and the presentation is highly variable. We present a case of ocular melanoma.

A 68-year-old man with a history of hypertension, osteoarthritis, and coronary artery disease came in after having worsening pain in multiple joints. Review of systems revealed worsening blurry vision and eye floaters. He denied eye pain or other associated complaints. He had no past history of any ocular pigmented lesions or history of skin cancer. Ophthalmology evaluation a few years earlier did not identify any abnormalities. Approximately 10 years prior to presentation, he did have LASIK surgery on both eyes. Subsequent ophthalmological evaluation showed an iris mass, elevated pressure, intra-retinal hemorrhages, and evidence of involvement in the choroid and conjunctivae. This was highly suspicious for iris melanoma of the right eye. He was started on intraocular pressure lowering medications and further workup was initiated. Biopsy confirmed the diagnoses of choroidal melanoma with an iris mass measuring 1 mm radially by 4 mm circumferentially. The mass extended posteriorly and involved well over half his iridocorneal angle resulting in very high intraocular pressure. A metastatic workup was done and was negative at the time. He underwent successful enucleation surgery with prostheses placement. Patient did well until about 1.5 years later when he was found to have multiple liver lesions suggestive of metastasis. This is currently being further evaluated.

No current guidelines exist for the screening of primary ocular melanoma as well as for screening for metastasis in those already diagnosed. Unfortunately, up to 50% of patients with ocular melanoma develop metastases. This case opens the discussion of needing current guidelines for screening and better surveillance in ocular melanomas. It highlights the importance of looking into screening using genomics and also developing targeted therapies, as well as focusing on immunotherapies for these cases.

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Fed Pract. 2016 September;33 (supp 8):18S
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