Varaha Sairam Tammisetti, MD

Venkata Katabathina, MD

Anil K Dasyam, MD

MDCT AND MR IMAGING OF ABDOMINAL MANIFESTATIONS OF HEMATOLOGICAL

CONDITIONS

DISCLOSURE •  None of the authors have relevant financial disclosure.

LEARNING OBJECTIVES

•  Illustrate the imaging appearances of varied abdominal manifestations of hematologic conditions of different etiologies

•  Discuss and illustrate typical abdominal imaging manifestations of some of these conditions and their differential

•  HEMOGLOBINOPATHIES AND ANEMIA •  Sickle cell anemia

•  DISORDERS OF COAGULATION

•  Hemophilia •  Paroxysmal nocturnal hemoglobinuria

•  Thromboembolic disease •  Ruptured mass

•  Spontaneous bleeding •  HELLP syndrome •  Hemorrhagic pancreatitis

•  Complications from anticoagulant therapy such as bilateral adrenal hemorrhage, suburothelial hemorrhage

•  DISORDERS OF IRON OVERLOAD •  Primary Hemochromatosis •  Secondary hemochromatosis and

Hemosiderosis •  INFECTIONS

•  Epstein Barr Infection •  AIDS

•  NEOPLASMS •  Lymphoproliferative disorders •  Leukemia •  Tumors of endothelial origin

OUTLINE

•  Hemolytic anemia

•  Splenic autoinfarction, abscesses rarely

•  Hepatic, renal and bone marrow iron deposition

•  Cholelithiasis and choledocholithiasis with pigmented stones

•  Renal cortical hemosiderin deposition, Papillary necrosis, Medullary carcinoma in the kidney

•  Role of imaging is to detect complications or cause for pain/hematuria

SICKLE CELL ANEMIA Common and Typical abdominal imaging manifestations

Anemias and Hemoglobinopathies:

•  Splenic autoinfarction •  Diffuse bony infarcts with sclerosis and typical H-shaped vertebral bodies

•  Findings: Marked decrease in T2 signal intensity in liver, bone marrow, spleen with autoinfarction and also decreased T2 signal in the renal cortex indicating renal cortical hemosiderin deposition

•  Hemosiderosis from Sickle Cell disease

•  Recurrent priapisms resulting in fatty replacement in corpora cavernosa

•  Infiltrative central right renal mass in a patient with history of Sickle Cell disease.

•  Hyperattenuating clots/blood in the pelvicalyceal system corresponding to hematuria

•  Diagnosis: Medullary Carcinoma

•  Thromboembolic disease

•  Hepatic veno-occlusive disease

•  Venous thrombosis such as from Nephrotic syndrome or postpartum ovarian vein thrombosis

•  Hemophilia

•  Paroxysmal nocturnal hemoglobinuria

•  Ruptured mass

•  Spontaneous bleeding

•  HELLP syndrome

•  Complication from Pancreatitis

•  Complications from anticoagulant therapy such as bilateral adrenal hemorrhage, suburothelial hemorrhage

DISORDERS OF COAGULATION:

Bleeding disorders/tendencies Hypercoagulability

HEMOPHILIA

•  Secondary to bleeding: Intramural hemorrhage/hematoma in bowel, mesenteric hematoma, Pseudotumor

•  Transfusion related diseases: HIV and Hepatitis C and malignancies related to those

•  ROLE OF IMAGING: •  To detect manifestations

secondary to bleeding •  Malignancies from

transfusion related disorders

Common and Typical abdominal imaging manifestations

Disorders of Coagulation

•  Duodenal hematoma and hemoperitoneum

•  Mesenteric hematoma simulating a mesenteric mass

•  Jejunal submucosal hemorrhage

2012 1 year later

•  Right retroperitoneal “pseudotumor” from old organized hemorrhage

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA •  A myelodysplastic, hematopoietic stem-

cell disorder characterized by increased sensitvity to complement-mediated intravascular hemolysis

•  Renal failure from cortical hemosiderin deposition

•  Hypercoagulability resulting in venous thrombi and also Budd-Chiari Syndrome with liver failure. Important to recognize the etiology as these are poor candidates for liver transplant

Common and Typical abdominal imaging manifestations

Disorders of Coagulation

•  60 yo male with new onset liver failure •  Coronal T2WI, Axial Arterial and Portal venous phase images of Liver •  Findings: Thrombus in Intrahepatic IVC, Hepatic vein thrombosis, hepatic parenchymal

infarcts •  Diagnosis: Budd-Chiari syndrome

•  Same patient •  Hypointense cortex on Coronal T2WI, Drop in signal within renal cortex on Axial In-phase images of Kidneys •  Findings: Cortical hemosiderosis •  Diagnosis: Budd-Chiari syndrome secondary to Paroxysmal Nocturnal Hemoglobinuria

RUPTURED MASS

•  Numerous hepatic adenomas on this patient who is on oral contraceptives. Recent rupture of an adenoma in right lobe with large intraparenchymal and subcapsular hematoma

Disorders of Coagulation

•  Bilateral Renal Angiomyolipomas: Ruptured AML on left with large perirenal hematoma

•  Ruptured pancreatic tail Solid Pseudopapillary neoplasm with large surrounding hematoma in LUQ and hemoperitoneum

•  Variety of hypervascular tumors in the abdomen can result in rupture and hemoperitoneum presenting to the ER

•  Important to recognize the source of hemorrhage for appropriate management

•  Common and Typical abdominal imaging manifestations

SPONTANEOUS BLEEDING

•  Spontaneous right renal subcasular hematoma

•  Defined as presence of hemorrhage or hematoma from a nontraumatic or noniatrogenic cause

•  Appearance and therefore management is variable by location and etiology

Disorders of Coagulation

•  Large spontaneous left rectus sheath hematoma with active extravasatiion (yellow arrow)

HELLP SYNDROME

•  Pregnancy related complication with hemolysis, elevated liver enzymes, low platelets (HELLP)

•  Life threatening complication is hepatic hemorrhage and rupture

•  Prompt diagnosis and awareness is required for appropriate management

Common and Typical abdominal imaging manifestations

Disorders of Coagulation

•  History: 29 yo female with history of pregnancy complicated by Eclampsia, acute liver and renal failure.

•  Findings: Large subcapsular hematoma about the liver with irregularity of the liver suggesting source of hematoma and also large T1 hyperintense hemoperitoneum

•  Diagnosis: HELLP Syndrome

T2WI 3D T1WI Precon

T2WI 3D T1WI Post con

T2WI

HEMORRHAGIC PANCREATITIS AND COMPLICATIONS •  Spontaneous hemorrhage in necrotizing

pancreatitis can occur from erosion of vasculature by necrosis or from rupture of a pseudoaneurysm or varices

•  Hemorrhage can occur within the pancreatic parenchyma, fluid collections, or the gastrointestinal tract

•  1%–5% incidence with mortality rates of 34%–52%

•  Splenic artery, portal vein, splenic vein, and other smaller peripancreatic vessels are the most common sources of bleeding

Disorders of Coagulation

•  Hematoma from ruptured splenic artery pseudoaneurysm – Patient with pancreatitis

•  Hemorrhagic pancreatitis with large hyperattenuating peripancreatic blood from spontaneous hemorrhage

COMPLICATIONS FROM ANTICOAGULANT THERAPY

•  Diagnosis: Suburothelial hemorrhage in renal pelvis resulting in hematuria, an expected complication with Coumadin

•  Diagnosis: Bilateral adrenal hemorrhage on a patient with cortical sinus venous thrombosis on Coumadin

•  Can involve multiple sites and multiple compartments

•  Abdominal wall – Rectus sheath and Iliopsoas

•  Visceral: Perirenal, intrarenal, adrenal, bowel hematomas

•  Renal- Suburothelial hemorrhage, clots in collecting system

•  Hemoperitoneum with hematocrit level

Disorders of Coagulation

•  Thromboembolic etiologies manifest as multi organ infarcts

•  Venous thrombosis such as from Nephrotic syndrome and from ovarian vein thrombosis in postpartum state can result in potential pulmonary emboli

HYPERCOAGULABILITY

Conditions and manifestations

•  Postpartum female with right flank pain and fever •  Hyperattenuating right ovarian vein thrombosis •  Diagnosis: Right ovarian vein thrombosis not extending into IVC

PRIMARY HEMOCHROMATOSIS

•  Decreased T2 signal in the Liver, spleen (not pancreas) and bone marrow •  Known patient with Hereditary Hemochromatosis

Hereditary autosomal recessive results in increased absorption of dietary iron excess iron deposited in the liver, pancreas, thyroid, heart and pituitary gland leads to cellular damage, organ dysfunction and malignancy If untreated may progress to cirrhosis, HCC, DM, cardiac dysfunction

SECONDARY HEMOCHROMATOSIS AND HEMOSIDEROSIS •  Secondary hemochromatosis

•  Parenteral administration of iron (such as multiple transfussions)

•  iron predominately deposited in the reticuloendothelial system (spleen and liver)

•  Total body iron may be 50-60 g Hemosiderosis: •  Increased iron deposition without organ damage •  Usually seen with body iron stores of 10-20 g

•  Findings: Marked decrease in T2 signal intensity in liver, bone marrow, spleen with autoinfarction and also decreased T2 signal in the renal cortex indicating renal cortical hemosiderin deposition

•  Hemosiderosis from Sickle Cell disease

EPSTEIN BARR VIRUS AND HIV INFECTION •  Can result in lymphoproliferative

malignancies with immunospuression including the post transplant setting

•  Bilateral adrenal masses, retroperitoneal adenopathy, left renal mass.

•  AIDS related Diffuse Large B cell Lymphoma

LYMPHOMA •  Primary and secondary

•  Nodal and extranodal Non-Hodgkin lymphomas including AIDS related lymphomas can involve multiple visceral organs along with lymph nodes.

•  Lymphadenopathy, visceral organ masses, peritoneal carcinomatosis, bowel wall thickening/mass if involving bowel, rarely can also present with GI bleeding •  Bilateral adrenal masses, retroperitoneal Diffuse

Large B-cell Non-Hodgkin’s Lymphoma with retroperitoneal adenopathy, bilateral renal masses, peritoneal carcinomatosis

•  Distal ileal aneurysmal dilation with wall thickening and mesenteric nodal mass consistent with primary small bowel lymphoma

Neoplasms

LEUKEMIA

•  Bleeding related complications with bleeding from multiple sites including bowel

•  Neutropenic fever with infectious enteritis

•  Granulocytic sarcoma or Chloroma is rare solid tumor can mimic abscess or hematoma. Important to recognize as they respond favorably to radiation than to chemotherapy

•  AML with thrombocytopenia and vaginal bleeding with uterine hematoma

•  Acute Myelogenous Leukemia (AML) with chloroma in Cervix

•  AML with neutropenic fever: Small bowel wall thickening proven to be infectious enteritis

Neoplasms

Sag T2WI of Uterus DWI

Sag Noncon CT

TUMORS OF ENDOTHELIAL ORIGIN

•  Diffuse hepatosplenic angiosarcoma •  Giant cavernous hepatic hemangioma

•  Hemangioma, Hemangioendothelioma and angiosarcoma are tumors of endothelial origin

•  Primary hepatic angiosarcoma exhibits a spectrum of appearances that reflect its varied pathologic features. Dominant mass or multiple masses or infiltrative mass can be seen on imaging.

Neoplasms

•  Right Renal Pecoma

•IATROGENIC: PSEUDOANEURYSM, HEMATOMA

•  RLQ Transplant perirenal hematoma – Postoperative day 0

SUMMARY AND CLINICAL IMPLICATIONS •  A variety of hematologic disorders have varied abdominal imaging

manifestations, some conditions have non-specific findings, some conditions have typical findings.

•  Multiple organs may be affected at the same time, for example paroxysmal nocturnal hemoglobinuria can present with Budd Chiari syndrome and renal cortical hemosiderosis, each of the findings have a differential but the combination of findings would help narrow the differential.

•  In summary, abdominal manifestations of hematological conditions are widely varied and familiarity of the abdominal manifestations of these conditions would allow Abdominal Radiologists to pinpoint to a diagnosis when the findings are typical or narrow the differential when findings are non-specific.

•  Abdominal manifestations of hematological conditions can be caused by a wide variety of etiologies and clinical management is ultimately dictated by the underlying etiology and pathology.

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Varaha.S.Tammisetti@uth.tmc.edu